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Atlas of Hair Pathology PDF
Atlas of Hair Pathology PDF
CLINICAL CORRELATIONS
An Atlas of HAIR
PATHOLOGY WITH
CLINICAL
CORRELATIONS
LEONARD C.SPERLING, MD, COL, MC, USA
Professor of Dermatology and Pathology
Chair, Department of Dermatology
Uniformed Services University
Bethesda, MD, USA
Contents
Preface
vi
Acknowledgements
vii
1.
2.
32
3.
37
4.
46
5.
50
6.
Clinical correlation
61
7.
65
8.
Androgenetic alopecia
68
9.
Telogen effluvium
81
10.
Trichotillomania
87
11.
Traction alopecia
94
12.
101
13.
105
14.
Alopecia areata
109
15.
Syphilitic alopecia
139
16.
147
17.
155
18.
163
19.
Lichen planopilaris
181
20.
193
21.
194
22.
207
23.
214
24.
224
25.
Tufted folliculitis
232
26.
Tinea capitis
234
27.
244
28.
249
Index
267
Preface
Acknowledgements
CHAPTER 1
Normal hair anatomy and architecture
The histological findings in many forms of hair loss are subtle, and an accurate
diagnosis depends on distinguishing abnormal from normal follicular
architecture. The word architecture refers to the anatomy of individual hair
follicles as well as the number, size and distribution of follicles within a biopsy
specimen.
SIZE OF HAIRS: TERMINAL,
INDETERMINATE,VELLUS
Long, thick hairs with bulbs in the fat are called terminal hairs. Vellus hairs are
thin, short and often hypopigmented, with bulbs located in the upper portion of
the dermis. Indeterminate hairs are intermediate in size between terminal and
vellus hairs. Hair shaft diameters can be readily measured in transverse section;
terminal hairs are thicker than 0.06 mm and vellus hairs are less than 0.03 mm. A
vellus hair can be readily identified by simple inspection, since its inner root
sheath will be as thick as or thicker than the shaft (Figures 1.1 and 1.2).
PHASE OF HAIR CYCLE: ANAGEN,
CATAGEN,TELOGEN
Every follicle, regardless of size, can be found in one of three phases of the hair
cycle. The anagen phase is the active growing period, and lasts weeks to years,
depending on the size and site of the hair. For human terminal scalp hair, anagen
lasts between 2 and 7 years. Catagen is a brief transitional phase between anagen
and telogen, and lasts about 23 weeks. The telogen phase lasts about 100 days,
at the end of which the shaft is shed. Depending on the individual, between 85
and 100% of terminal scalp hair is in the anagen phase at any given time; 015%
is in the telogen phase and only about 1% is in the catagen phase. The percentage
of terminal telogen hairs present (usually based on a lock of hairs forcibly
plucked from the scalp) is called the telogen count. Average telogen counts
are in the range of 613%, and a count greater than 20% is abnormal.
Figure 1.1 Terminal versus vellus hairs. Two terminal hairs and a single vellus hair,
sectioned at the level of the mid-dermis. This specimen was obtained from a normal adult
scalp. Original magnification 200
Figure 1.2 Terminal versus vellus hairs. Two terminal hairs and a single vellus hair,
sectioned at the level of the infundibulum (upper dermis). A sweat duct (lower left corner)
is present for comparison. Original magnification 200
Figure 1.3 Terminal anagen hair bulb, vertical section. The bulbs of terminal hairs are
usually located in the superficial fat or deep dermis. The basophilic (and often pigmented)
hair matrix surrounds the dermal papilla (P), whose stalk (S) is continuous inferiorly with
the fibrous root sheath. Original magnification 200
from the center of the follicle and moving outward, the layers are the hair shaft
medulla, the hair shaft cortex, the cuticular layer, Huxleys layer of the inner
root sheath, Henles layer of the inner root sheath, the glassy (vitreous) layer and
the fibrous root sheath. The cuticular layer is composed of the inter-locking
flattened cells of the hair shaft cuticle and the inner root sheath cuticle. These
cells are so tightly inter-locked that they appear to form a single anatomical layer
(Figures 1.5 and 1.6).
Moving toward the skin surface, the next zone is the isthmus. The inferior
landmark for the isthmus is the insertion of the arrector pili muscle into the
fibrous root sheath of the follicle. The superior landmark for the isthmus is the
entrance of the sebaceous duct into the follicular canal. The isthmus is an
important transitional zone of follicular keratinization. In the mid-portion of the
isthmus, the inner root sheath desquamates, resulting in a separation between the
hair shaft and the follicular wall. At this point, the cells of the outer root sheath
Figure 1.4 Terminal anagen hair bulb, transverse section. Original magnification 400
begin to cornify without the formation of a granular cell layer. This is called
trichilemmal keratinization (Figures 1.7 and 1.8).
The uppermost zone of the follicle is the infundibulum, bounded inferiorly by
the entry of the sebaceous duct. Cornification of the outer root sheath occurs with
the formation of a granular cell layer, similar to the epidermal surface (Figures
1.9, 1.10 and 1.11).
The epithelium adjacent to the insertion of the arrector pili muscle (the bulge)
is the putative site of the follicular stem cells. Although an actual anatomical
bulge is evident in rodent follicles, human follicles do not usually have an
obvious protuberance of epithelium at this site. Special histochemical stains
directed against antigens such as cytokeratin 15 are required to identify the stem
cells (Figures 1.12 and 1.13).
Hair shaft size and shape differs between racial groups. The hair shafts of
African Americans tend to be elliptical or kidney-bean-shaped (Figure 1.14), and
are situated eccentrically within the epithelium of the follicle. Caucasian hair
shafts tend to be circular or slightly oval, and are usually located directly in the
center of the follicle.
Catagen hair anatomy
Each terminal anagen hair on the scalp grows for about 27 years, depending on
the individual. At the end of that time, the hair shaft ceases active growth and
enters a brief catagen phase. Within just a week or two, the entire anatomy of the
follicle changes. At the beginning of catagen, the hair matrix disappears and is
replaced by a thin rim of epithelial cells surrounding the hair papilla. These
epithelial cells along with an overlying homogeneous column of epithelial cells
demonstrate nuclear pyknosis. The epithelium of the lower follicle is undergoing
disintegration by way of apoptosis (programmed cell death). As these epithelial
changes occur, the vitreous (or glassy) layer markedly thickens, so that it
becomes a prominent structure. The fibrous root sheath also thickens. As the
catagen phase progresses over a 23-week period, the hair papilla follows the
Figure 1.5 Terminal anagen hair, suprabulbar zone, vertical section. Just above the bulb,
the various layers of the anagen hair can be identified: medulla (M) if present, cortex (C),
cuticular layer (Cu), inner root sheath (IRS), outer root sheath (ORS), vitreous/glassy layer
(V) and fibrous root sheath (F). Original magnification 400
disintegrating epithelial column upwards into the dermis, and the papilla
eventually comes to rest just below the bulge zone (attachment of arrector pili
muscle).
As the epithelial column moves upward, a collapsed fibrous root sheath is left
behind. This collapsed structure is called the stela (or stele; plural, stelae),
derived from the Greek word for pillar. The stela is also referred to as the
follicular streamer. In this text, the terms stela and streamer are considered
to be synonymous.
Just above the epithelial column of the catagen hair, an expanded mass of
epithelium forms the presumptive club hair. Early in catagen the cells of the
presumptive club are still nucleated, but the nuclei disappear as the club begins
to cornify from the center outward (Figures 1.151.22).
Figure 1.6 Terminal anagen hair, suprabulbar zone, transverse section. Original
magnification 400
Figure 1.7 Isthmus of a terminal anagen hair. This zone is demarcated by the insertion of
the arrector pili muscle below and the opening of the sebaceous duct above. Original
magnification 100
Figure 1.8 The inner root sheath (IRS) abruptly desquamates in the mid-portion of the
isthmus. Above this, the outer root sheath (ORS) cornifies without the formation of a
granular cell layer. Original magnification 400
Figure 1.9 Terminal anagen hair, infundibulum,vertical section. The outer root sheath
cornifies with the formation of a granular cell layer. The space between the hair shaft and
follicular wall is filled with desquamated keratinocytes, sebum, bacteria, pityrosporon and
cosmetics. Much of this debris is removed during processing. Original magnification
400
bulb is not cornified, it is soft and can take on a bent or hockey stick
configuration. Just above the bulb, portions of both an inner and an outer root
sheath can be identified. Unfortunately, the outer root sheath or both root sheaths
are often left behind in the dermis, an artifact of plucking. This gives the hair a
dysmorphic (not dystrophic) appearance (Figures 1.291.31).
Catagen pull/pluck findings
Like anagen hairs, catagen hairs are seldom gently pulled from the scalp. They
are sometimes found when a lock of hair is forcibly plucked from the scalp.
Plucked catagen hairs closely resemble telogen hairs, except that a clear, noncornified sac surrounds the club, and a tail of soft, clear tissue lies below the
bulb. This tail represents the degenerating epithelial column that lies between
the hair papilla and cornifying club (Figure 1.32).
Figure 1.10 Terminal anagen hair, infundibulum, transverse section. Section through the
lower infundibulum showing a granular cell layer. Original magnification 400
Figure 1.11 Terminal anagen hair, infundibulum, transverse section. The infundibula of
two normal, separate follicles have merged into a common infundibulum containing two
hair shafts. This is often a normal finding, especially in African-American patients, as in
this case. Original magnification 200
Figure 1.12 Insertion of the arrector pili muscle into the follicle (bulge zone) in the
anagen phase. This portion of the follicle is home to the follicular stem cells, and is a
landmark demarcating the lower portion of the isthmus. Original magnification 400
center, and the hemostat is released. A few drops of immersion oil are dripped
onto the bulbs and a second glass slide or cover slip is placed on top. If the hair
is kinky or curly, the slides may need to be taped together so that the hairs lie
flat.
In theory, interpretation of the trichogram should be simple. The goal is to
obtain a telogen count, the percentage of telogen hairs represented in the
sample. However, considerable artifact may be introduced by the act of
plucking, and the anatomy of anagen hair bulbs may be badly distorted, making
some of them dysmorphic and difficult to identify. Hairs with bulbs that have
cleanly snapped off (as if cut) are counted as anagen hairs, as are bulbs that show
remnants of an outer or inner root sheath. A pigmented and bent bulb in the
absence of root sheaths can also be counted as an anagen hair. In this case, the
cuticle above the bulb will be ruffled (Figures 1.351.37).
BIBLIOGRAPHY
Headington J.Transverse microscopic anatomy of the human scalp. Arch Dermatol 1984;
120:44956
Lyle S, Christofidou-Solomidou M, Liu Y, Elder DE, Albelda S, Cotsarelis G. The C8/
144B monoclonal antibody recognizes cytokeratin 15 and defines the location of
human hair follicle stem cells. J Cell Sci 1998; 111:317988
Solomon A. The transversely sectioned scalp biopsy specimen: the technique and an
algorithm for its use in the diagnosis of alopecia. Adv Dermatol 1994; 9:12757
Sperling L.Hair density in African Americans. Arch Dermatol 1999; 135:6568
Sperling LC, Hair anatomy for the clinician (CME). J Am Acad Dermatol 1991; 25:117
Sperling LC, Lupton GP. The histopathology of non-scarring alopecia. J Cutan Pathol
1995;22:97114
Figure 1.13 The bulge zone, as seen with immunohistochemical markers for cytokeratin
15 and smooth muscle actin. The dark red staining of the outer root sheath, found in the
vicinity of the attachment of the arrector pili muscle, indicates the presence of cytokeratin
15. Cytokeratin 15 is a marker of the bulge zone. The brown staining of the arrector pili
muscle indicates the presence of smooth muscle actin. Photomicrograph courtesy of
George Cotsarelis, MD. Original magnification 100
Figure 1.14 Transverse section of a terminal anagen hair from an African-American. The
hair shaft is elliptical or kidney-bean-shaped, and is often eccentrically placed within the
surrounding epithelium. Original magnification 400
Figure 1.15 Terminal catagen hair, bulb, transverse section. A thin rim of pale-staining
epithelial cells, many with pyknotic nuclei, surround the hair papilla (P). The vitreous
layer (V) is markedly thickened. Original magnification 400
Figure 1.16 Terminal catagen hair, suprabulbar, transverse section. Numerous pyknotic
nuclei are present, and the markedly thickened vitreous layer (V) is clearly seen. Original
magnification 400
Figure 1.17 Terminal catagen hair, suprabulbar, transverse section, periodic acid-Schiff
(PAS) stain. PAS staining highlights the thickened vitreous layer. The fibrous root sheath
(F) is also much thicker than in an anagen hair. Original magnification 400
Figure 1.18 Terminal catagen hairs, suprabulbar, transverse sections. The follicle on the
right is sectioned just below the presumptive club hair where the epithelial column is
widest. The other is sectioned through the presumptive club, which is just beginning to
cornify centrally Original magnification 200
Figure 1.19 Lower half of a terminal catagen hair, vertical section. The bulb (B;
corresponding to Figure 1.15), disintegrating epithelial column (EC; corresponding to
Figure 1.16) and presumptive club (PC; corresponding to Figure 1.18, left side) can be
seen. Original magnification 200
Figure 1.20 Vertical section of the stela (or streamer) below the bulb of a catagen hair.
The stela is the collapsed fibrous root sheath found below hairs in the catagen/telogen
phase or hairs that have miniaturized. Original magnification 200
Figure 1.21 Vertical section of the stela, as in Figure 1.20. Original magnification 400
Figure 1.22 Transverse section of three stelae, demonstrating their roughly circular shape
and numerous small vascular spaces. Original magnification 400
Figure 1.23 Bulb of early telogen hair, vertical section. The hair papilla is seen as a tight
cluster of nuclei lying just below the nipple-like secondary hair germ (SHG; the telogen
germ unit, seen also in Figure 1.24). Original magnification 400
Figure 1.24 Secondary hair germ (SHG) or telogen germ unit, transverse section. Just
below this structure lies the hair papilla. Just above lies the cornifying club of the
telogen hair (if it has not yet been shed). A terminal anagen hair (A) is nearby for
comparison. Original magnification 400
Figure 1.25 Transverse section of an early telogen hair bulb. The cornifying clubs
serrated rim interdigitates with the surrounding envelope of outer root sheath epithelium.
Original magnification 400
Figure 1.26 Bulb of a late telogen follicle, transverse section. The cornified club now fills
most of the width of the follicle, with only a thin rim of non-cornified epithelium
remaining. The club hair is now ready to be shed. Original magnification 400
Figure 1.27 Section through the bulbs of a terminal telogen hair and a vellus telogen hair.
Original magnification 200
Figure 1.28 Newly growing anagen hair, extending down from the bulge zone. The
telogen club hair has not yet been shed. Original magnification 100
Figure 1.29 Bulb of forcibly plucked, terminal anagen hair. Original magnification 200
Figure 1.30 Suprabulbar zone of forcibly plucked, terminal anagen hair (IRS, inner root
sheath; ORS, outer root sheath; F, fibrous root sheath). Original magnification 200
Figure 1.31 Dysmorphic, plucked terminal anagen hair. Here the term dystrophic should
not be applied, because the loss of the root sheaths is not a growth defect but merely an
artifact of plucking. Original magnification 100
Figure 1.32 Forcibly plucked catagen hair. The tail-like remnant of the degenerating
epithelial column is found just below the presumptive club. Original magnification 200
Figure 1.33 Forcibly plucked early telogen hair. A noncornified epithelial sac surrounds
the cornifying club. Original magnification 200
Figure 1.34 Forcibly plucked or gently pulled late telogen hairs. The clubs are completely
cornified and the epithelial sac is gone. Original magnification 200
Figure 1.35 Forcibly plucked lock of hairsthe trichogram. In this field of 23 terminal
hairs, 21 anagen hairs and two telogen hairs are present. The telogen count is therefore 2/
23, or about 9%. Original magnification 40
Figure 1.36 Snapped-off shaft, an artifact of plucking. A telogen hair (upper hair) and a
snapped-off shaft are found in this field. When deeply rooted, terminal anagen hairs are
plucked, a clean transverse break in the shaft, as seen here, is sometimes the result. Shafts
such as these can be counted as anagen hairs. Original magnification 200
Figure 1.37 Dysmorphic, terminal anagen hair, an artifact of plucking. Sometimes when
a terminal anagen hair is plucked, the entire shaft is removed but the root sheaths are left
behind in the scalp. The pigmented, bent root (often resembling a hockey stick) and the
ruffling of the cuticle of the proximal shaft identify such a hair as an anagen hair. A
similar hair is seen in Figure 1.31. Original magnification 200
CHAPTER 2
Specimen acquisition, handling and
processing
ACQUISITION
Selecting the biopsy site is the most difficult and important part of the process.
The most fruitful site will vary depending on the disease, and often the clinician
is uncertain of the diagnosis. Sampling the center of a lesion of alopecia areata or
trichotillomania would be appropriate. Sampling the bald center of a lesion of
scarring alopecia is seldom useful, and the active peripheral margin would be a
more suitable target. Compounding the difficulty of site selection is sampling
error. Clinicians cannot see below the surface of the skin, and even the most
experienced physician may choose an unrewarding spot. If a recently involved
area of scalp showing early clinical changes is selected, the diagnostic yield will
be higher. Highly inflamed sites (pustules or papules) are often very advanced
lesions and are frequently non-diagnostic. Multiple separate specimens chosen
from several sites in or around the lesion will increase the diagnostic yield.
However, clinicians may not have the luxury of obtaining multiple specimens.
In some cases the patients normal scalp can serve as a basis for comparison
with the abnormal scalp. For example, a specimen from the mid-occiput can help
establish a diagnosis of common balding in a patient with hair loss on the crown.
Once the site is selected, it should be anesthetized with lidocaine with
epinephrine (adrenaline). A generous amount of anesthetic (13 ml) should be
injected into the deep dermis and superficial fat, and allowed to act for 1530
minutes before the biopsy is performed. This will minimize bleeding.
The blade of the punch biopsy tool should extend through the dermis down
into the fat, so that intact bulbs of deeply rooted terminal hairs can be removed.
A 4-mm biopsy wound can be easily closed with 30 suture because the
needle can traverse the wound in a single pass. A suture color that contrasts with
the patients hair will assist in suture removal 1 week after the biopsy is
performed.
HANDLING
Once obtained, the scalp biopsy specimen should be allowed to fix in formalin
for at least 24 hours before sectioning. Biopsy specimens obtained for direct
immunofluorescence testing should of course be placed in the appropriate
transport solution.
PROCESSING
The required tools include a sharp blade, a blade holder, a pair of fine-toothed
forceps, marking ink, a cotton-tipped applicator and the standard plastic
specimen cassette. Sponges should be used inside the cassette to prevent the thin
slices of tissue from escaping. The sharpest blade for the job is a disposable,
flexible shaving blade (blue blade). These can easily be snapped in half for
economy and safety (Figure 2.1). The author prefers to use red marking ink, but
Figure 2.1 Tools for performing transverse sections. The flexible shaving blade has been
snapped in half
Figure 2.2 The specimen is placed on its side like a cylindrical loaf of bread, and is gently
stabilized with forceps. A flexible, disposable shaving blade is ideal for taking the slices
any color will do. A specially colored cassette will alert the histology technician
that embedding must be performed in a particular way.
There are several ways to slice the tissue into transverse sections. The
technique used by Headington and Whiting involves a single transverse slice
about 1 mm below the epidermal surface. Both cut sides of the specimen are
embedded down in the cassette. As the microtome cuts deeper into the tissue
block, the sections become progressively more superficial in one half of the
specimen and deeper in the other. Simply sectioning deeper into the block allows
one to obtain sections as superficial or as deep as required.
An alternative method is that of Frishberg and Sperling. One treats the biopsy
specimen like a cylindrical loaf of bread, and cuts it into three or four slices
(Figures 2.2 and 2.3). The slices are about 1 mm thick. The deep surface of
each slice is inked (Figure 2.4) and the inked sides are placed down in the
cassette. Once the ink has been removed by the microtome, a single section is
taken. In this way, the specimen is sampled at several different depths
(Figure 2.5). Because only a single section is needed to view multiple levels on a
Figure 2.3 The specimen is cut into three or four slices, which are placed with the cut
surfaces up, so that they are ready for inking
Figure 2.4 The inked slices are placed in a cassette, packed between sponges. They will
be embedded in paraffin, ink side down
single slide, the remainder of the tissue in the block is available for recuts and
special stains.
Yet another but more tedious method involves embedding the epidermal and/or
fat end down in the cassette, and taking multiple horizontal sections through the
entire specimen. Dozens of sections can be required to section through the entire
block, and the block is exhausted in the process. However, every possible level
of every follicle in the specimen can be carefully studied, and this technique is
useful for research purposes.
BIBLIOGRAPHY
Frishberg DP,Sperling LC,GuthrieVM. Transverse scalp sections: a proposed method for
laboratory processing. J Am Acad Dermatol 1996; 35:2202
Headington J. Transverse microscopic anatomy of the human scalp. Arch Dermatol 1984;
120:44956
Figure 2.5 The final product is a single slide containing disks of tissue from multiple
levels (superficial to deep)
Whiting DA. Diagnostic and predictive value of horizontal sections of scalp biopsy
specimens in male pattern androgenetic alopecia. J Am Acad Dermatol 1993; 28:
75563
Whiting DA, Howsden FL Color Atlas of Differential Diagnosis of Hair Loss. Cedar
Grove, NJ: Canfield Publishing, 1996
CHAPTER 3
Evaluating and describing transverse
(horizontal) sections
Figure 3.1 A vertical section bisecting the specimen through plane X would sample
only four follicles out of a total of 28 contained in the specimen. A section through plane
Y would not sample any hairs
(2) Are follicular units evenly spaced, or are there blank spots with an absence
of follicles, suggesting scarring or severe miniaturization (Figures 3.2 and
3.3)?
(3) Do most follicular units contain two to six follicles, with large follicles
outnumbering small follicles (Figure 3.4)
(4) Are a normal number (> 85%) of the terminal follicles in the anagen phase
(Figure 3.5)?
(5) Do any follicles show incomplete or distorted anatomical features
(Figure 3.6)?
(6) Is inflammation present, and at what level of the follicle (bulb, suprabulbar,
isthmus, infundibulum)?
(7) Is perifollicular fibrosis present; have individual follicles been entirely
replaced by connective tissue (true follicular scars; Figure 3.7)?
(8) What is the total number of viable follicles in the specimen? The total
number of terminal anagen hairs? The total number of vellus hairs?
The information gleaned from this evaluation will be used to compose the
microscopic description. Interpreting the data and arriving at a final diagnosis is
more challenging, and the major purpose of this text is to assist in this process.
With few exceptions, the diagnosis of most forms of alopecia hinges on a
constellation of findings and not on a single finding. The group of findings
typical of each disease is described in the chapters devoted to particular entities.
THE NORMAL SCALP IN TRANSVERSE SECTION
The architecture of a transversely sectioned biopsy specimen is quite
predictable, and numerical data from the study of normal scalp specimens are
available. Most data are based on 4-mm punch biopsy specimens, which can be
considered the standard. A specimen 4 mm in diameter samples a surface area
Figure 3.2 Example of blank spots in the follicular architecture, suggesting loss of
follicles. The zones of follicular loss are quite obvious because of the tinctorial difference
between follicular and non-follicular dermal collagen. Original magnification 40
Figure 3.3 Multiple follicles are missing and have been replaced by normal-appearing
dermal collagen. Superficial levels assessed on this specimen confirmed a marked
reduction in total follicular numbers. Original magnification 100
of 12.6 mm2 = r2. This formula can be used to compare data from punch
biopsies larger or smaller in diameter with those of a 4-mm punch. For example,
a 3-mm punch biopsy specimen has a surface area of 7.07 mm2.
In a 4-mm punch biopsy specimen from a Caucasian, there should be about 33
terminal hairs in a transverse section through the deep dermis (Figure 3.8).
Sectioning through the upper dermis will reveal about five additional vellus hairs
(Figure 3.9). The average scalp biopsy specimen will therefore contain about
38 follicles. Considerable individual variation exists and the range of normal is
quite broad; total hair counts as low as 19 and as high as 59 have been recorded.
On average, two of the terminal hairs will be in the telogen phase, and 31 will be
in the anagen phase. The telogen count (number of terminal telogen hairs divided
by the total number of terminal hairs) will therefore be about 2/33=6%.
Considerable individual variation exists for telogen counts among normal
individuals, and figures within a range of 015% (based on histological sections)
can be considered normal.
Figure 3.4 Transverse section through the upper dermis of a specimen from a normal
scalp. Two follicular units are shown, the one on the left containing one terminal and two
vellus hairs, and the one on the right containing three terminal hairs. Although the first
unit may not appear normal because vellus outnumber terminal hairs, all the follicular
units need to be assessed to make a conclusion about overall normality. Original
magnification 100
Figure 3.5 In this field, only two of five terminal follicles (40%) are in the anagen phase.
This would be clearly abnormal if it were representative of the entire specimen. Original
magnification 100
On the average, black patients of African descent have fewer, although larger,
follicles than white patients (Figures 3.10 and 3.11). The average number of
follicles for African Americans, based on 4-mm biopsy specimens, is 21 (18
terminal and three vellus follicles) with an average telogen count of 7%. The
percentage of catagen and/or telogen hairs can prove to be an important
diagnostic feature in many forms of hair loss. Catagen and telogen hairs have
the same diagnostic significance, since all catagen hairs become telogen hairs
within a few weeks. For the sake of simplicity, these two phases can be grouped
together as the catagen/telogen phase. In this textbook, the term catagen/telogen
phase will be used to refer to hairs in either the catagen or the telogen phase. For
diagnostic purposes, it is not important to differentiate between catagen and
telogen hairs, but just to recognize a follicle as being in either phase. Catagen
Figure 3.6 Distorted follicular anatomy, in this case due to forcible plucking of the hair
shaft prior to taking the biopsy specimen. The shaft and a portion of the inner root sheath
are missing, and the epithelium has collapsed inward. Original magnification 400
Figure 3.7 True follicular scars, diagnostic of a scarring alopecia. In transverse section,
they appear as discrete, circular or oval structures composed of compact and concentric
collagen bundles. Original magnification 400
hairs can be included with the telogen hairs when determining the telogen count
(Figure 3.12).
In the superficial fat and lower dermis, home to the bulbs and suprabulbar
zones of terminal anagen hairs, the follicles are spaced apart fairly evenly.
However, in the mid- and upper dermis, groups of follicles become segregated into
follicular units. Each follicular unit contains about two to five terminal hairs and
zero to two vellus hairs (Figure 3.13). A shortcut for assessing numerical
normality is by looking at individual follicular units (Figure 3.14). If each unit
contains two to five terminal hairs (outnumbering vellus hairs), and most units
are similar, then the number and size of hairs are probably normal. As would be
expected, the number of terminal hairs per follicular unit is smaller in African
Americans than in Caucasians.
The average density of follicular units is about 1/mm2 surface area. Therefore,
a horizontal section 4-mm in diameter (12.6 mm2) will contain about 12
follicular units.
Figure 3.8 Transverse section from normal scalp skin (Caucasian) at the level of the
superficial fat. Only terminal follicles can be found at this level, and they are not yet
organized into follicular units. Original magnification 40
Figure 3.9 Transverse section from normal scalp skin (Caucasian) at the level of the
upper dermis. The bulbs of telogen hairs and vellus hairs can also be found at this level,
organized into follicular units. Original magnification 40
In the normal scalp, the terminal: vellus hair ratio should be 2:1 or greater. If
biopsy specimens from the crown and occiput of the same person are compared,
they should be similar in terms of follicular size, number and percentage of
anagen/telogen hairs. The architecture of scalp biopsy specimens should be
uniform over
Table 3.1 Template for a hair biopsy report
Accession number:
Date:
Patient name/age/sex/race:
Submitting physician:
Clinical impression:
Macroscopic description: biopsy diameter/location on scalp
(e.g. 4-mm punch biopsy, vertex of scalp):
Microscopic description of vertical sections:
Microscopic description of horizontal sections:
Figures 3.10 Transverse section from normal scalp skin (African American) at the level
of the deep dermis. Normally, only terminal and indeterminate follicles can be found at
this level, and they are not yet organized into follicular units.This specimen contained 16
total follicles. Original magnification 40
Figure 3.11 Another transverse section from normal scalp skin of an African American,
containing 22 follicles. These numbers would be regarded as low for a Caucasian but are
normal in African Americans. Original magnification 40
Terminal anagen hairs:
Terminal catagen hairs:
Terminal telogen hairs:
Telogen germinal units:
Vellus hairs:
Total hairs (terminal plus vellus, all phases):
Anagen : telogen percentages (e.g. 85% : 15%):
Terminal: vellus ratio (e.g. 3:1):
Follicular units:
Follicular stelae:
Lymphohistiocytic infiltrate:
Upper follicle:
Lower follicle/bulb:
Fibrosis:
Comments:
Figure 3.12 Transverse section through the level of the dermal-subcutaneous junction. In
this particular field, there are 12 terminal anagen hairs and seven terminal telogen hairs,
for a total of 19 terminal hairs. The telogen count for this field would therefore be 7/
19=37%. Original magnification 40
Figure 3.13 Normal follicular unit. This unit contains four terminal hairs and one vellus
hair. Original magnification 100
DIAGNOSIS:
Consultants:
Pathologists signature_________________
Figure 3.14 Two follicular units from normal scalp skin. One unit contains three terminal
anagen hairs and the other contains one terminal anagen and one vellus telogen hair. Even
within the same specimen, the number of hairs per follicular unit varies somewhat; so
several units need to be examined to arrive at a valid average. Original magnification
100
CHAPTER 4
Classif ication of hair disease
Various classification schemes for alopecia exist, but all are imperfect. Most
forms of alopecia demonstrate at least some overlapping clinical and histological
features. This overlap blurs the distinction between diseases, making
classification difficult. It would make the most sense to segregate diseases by
etiology, but the causes of many forms of hair loss are unknown, making it
difficult to group diseases with confidence.
CICATRICIAL (SCARRING) VS. NON-CICATRICIAL
ALOPECIA
The most common classification system divides the diseases into cicatricial
(scarring) and non-cicatricial (non-scarring) forms of alopecia. In this textbook,
the terms cicatricial and scarring will be considered to be synonymous and
will be used interchangeably. Cicatricial or scarring implies that follicular
epithelium has been replaced by connective tissue. However, in some cases of
alopecia, follicles seem simply to disappear without noticeable alteration in
tissue architecture. The broadest definition of scarring alopecia might include all
forms of alopecia in which hair follicles are permanently lost. In contrast, nonscarring alopecia is potentially reversible.
However, certain hair diseases demonstrate a biphasic pattern, where nonscarring hair loss is seen early in the course of the disease, and permanent hair
loss becomes apparent in the later stages of the disease (Figure 4.1). Examples
of diseases demonstrating this biphasic pattern include androgenetic alopecia,
alopecia areata and traction alopecia. These forms of alopecia are generally
considered to be non-scarring. However, after many years or decades of
continuous active disease, permanent dropout of follicles occurs.
CLASSIFICATION OF CICATRICIAL (SCARRING)
ALOPECIA
This classification is especially confusing and controversial. There are no
characteristic biological markers for most forms of scarring alopecia. We do not
know whether the clinical and histological features found in a given patient are
47
Figure 4.1 The biphasic form of alopecia. With the passage of time, follicles begin to
disappear permanently and histological specimens take on the appearance of a cicatricial
alopecia
Figure 4.2 Classification of scarring alopecia. The ovals in this diagram overlap for
several reasons. First, a diagnostic entity may share at least some clinical and/or
histological features with another form of cicatricial alopecia. Second, it is possible that
two different entities may some day prove to be variations of the same disease (this
concept is discussed in Chapter 23). Finally, the overlap of these distinct entities serves
as an admission that the conditions are poorly understood, and that their separation in this
classification is provisional. LPP, lichen planopilaris (FFA, frontal fibrosing alopecia is a
subset of LPP); CCLE, chronic,cutaneous lupus erythematosus;CCSA,central,centrifugal
scarring alopecia; AKN, acne keloidalis
This short list is a simplification of a longer list of confusing, vague and poorly
defined diagnostic terms that have been coined and used by various authors
during the past century. Notably absent from the above list are terms such as
pseudopelade, pseudopelade of Brocq, folliculitis decalvans, tufted folliculitis
and a variety of other more obscure terms. These entities are poorly defined, and
49
CHAPTER 5
Distinctive or critical histological features
and associated diseases
Figure 5.1 An example of normal hair density. A transverse section from the normal
(uninvolved) scalp of an African American woman with traction alopecia. The section
contains 22 follicles, slightly more than average. Original magnification 40
Figure 5.2 An example of decreased hair density from the same woman as in Figure 5.1.
A section from the zone of alopecia contains 11 follicles, a marked reduction compared to
her normal scalp. Original magnification 40
Telogen effluvium
Androgenetic alopecia
Temporal triangular alopecia
Traction alopecia
Figure 5.3 In this specimen from a patient with alopecia areata (and that in Figure 5.4), the
clinician took the biopsy from a bald spot, but normal or nearly normal numbers of
follicles were present. This can be explained by an increase in miniaturized hairs
producing no hair shaft. Original magnification 40
Figure 5.4 As in the patient in Figure 5.3, this specimen was taken from a bald spot of
alopecia areata, but normal or nearly normal numbers of follicles were present. In this
case it was because of a massive conversion to telogen hairs. Original magnification 100
Figure 5.5 Miniaturized hairs in a patient with temporal triangular alopecia. Average
hair shaft diameter is 0.02 mm. Original magnification 200
Figure 5.6 Normal hairs from the same patient as in Figure 5.5 (normal, perilesional
skin). Average shaft diameter is 0.12 mm. Original magnification 200
Figure 5.8 Higher power view of specimen shown in Figure 5.7. Original
magnification 100
Figure 5.9 In this patient with alopecia areata, the lower half of a follicle is affected by
lymphocytic inflammation. Original magnification 400
Figure 5.10 The upper half of the follicle in Figure 5.9. This half is spared lymphocytic
inflammation. Original magnification 400
Trichotillomania
Traction alopecia (acute)
Postoperative (pressure-induced) alopecia
Telogen effluvium
Alopecia areata
Androgenetic alopecia/hereditary balding (affected area only)
Figure 5.13 A section through the same follicle as in Figure 5.12, but at the level of
the dermal/fat junction. Inflammation spares the lower half of the follicle (but
premature desquamation of the inner root sheath is present). Original magnification
200
Figure 5.14 In this example of inflammatory scarring alopecia, there are blank spots
with residual inflammation, representing the sites of former follicles and follicular
units. The sebaceous glands have disappeared with the follicles. Original
magnification 40
Figure 5.15 The same specimen as in Figure 5.14. Original magnification 100
Figure 5.16 In this example of end-stage traction alopecia, all terminal hairs have
disappeared, but sebaceous glands are spared. Original magnification 100
Figure 5.17 In this specimen from a patient with central, centrifugal scarring alopecia
there is a follicle whose inner root sheath has desquamated well below the isthmus
(follicle at lower left). Normal follicles with intact inner root sheaths are present for
comparison. Original magnification 100
Figure 5.18 An enlargement of the abnormal hair found in Figure 5.17. Original
magnification 400
Trichotillomania
Acute traction alopecia
Pressure-induced alopecia
Alopecia areata
CHAPTER 6
Clinical correlation
The most important feature is the pattern of hair loss. Hair loss can be diffuse or
patterned. Patterned hair loss implies that the area of alopecia is confined to
one or several portions of the scalp, leaving at least a portion of the scalp
uninvolved. Truly diffuse hair loss suggests a uniform reduction in hair density
over all portions of the scalp. Telogen effluvium (Figure 6.1) is an example of
truly diffuse alopecia, with all areas of the scalp showing some thinning of the
hair. Patchy alopecia areata is one example of patterned alopecia. The various
patterns of thier loss are listed below.
PATTERNS OF HAIR LOSS
Truly diffuse hair losse.g., telogen effluvium, alopecia totalis in evolution,
chemotherapy/radiation therapy
Diffuse hair loss over crown/vertex with relative sparing of parietal/occipital
arease.g., androgenetic alopecia
Nearly total hair loss with loss of follicular ostia, centered on crown or vertex
e.g., central centrifugal scarring alopecia
Patches of hair loss with bizarre or geometric shapese.g., trichotillomania
Randomly scattered oval or circular patches of complete hair losse.g.,
alopecia areata
Figure 6.1 An example of truly diffuse hair loss (telogen effluvium). Hair thinning is
uniform over the crown, occiput and sides of the head
63
Figure 6.2 An example of patterned hair loss in a woman with androgenetic alopecia. The
crown is diffusely and symmetrically affected with relative sparing of the occiput as seen
in Figure 6.3
histiocytosis (histiocytosis X) of the scalp can cause dramatic scalp disease with
little effect on hair density.
BIBLIOGRAPHY
Sperling LC. The evaluation of hair loss. Curr Probl Dermatol 1996; 8:99136
Sperling LC, Mezebish DS. Hair diseases. Med Clin North Am 1998; 82:115569
CHAPTER 7
Senescent balding (senile alopecia)
Pure senescent alopecia is found in patients who boast a full head of hair well
into middle age, and who typically deny a family history of balding. Patients
with senescent alopecia note a very slow but steady, diffuse thinning of scalp
hair starting at age 50 and older. Women who complain of a marked degree of
thinning in the few years following menopause probably have a component of
androgenetic alopecia. Many (and perhaps most) patients with senescent alopecia
also have mild concomitant androgenetic alopecia, and the superimposed clinical
and histological features of common balding and senescent alopecia may be
impossible to separate.
Several authors have assessed the effect of aging on hair density by studying
the scalp surface (i.e., clinical rather than histological evaluation). The results of
these studies indicate that the density of hair follicles decreases steadily with
aging. There is little information available on the histological evaluation of scalp
biopsy specimens taken from normal individuals of various ages.
HISTOLOGICAL FINDINGS
The global impression when studying transverse sections is that there is only a
slight numerical reduction in follicles that are otherwise normal. Compared to
normal (or youthful) scalp, more follicles are in the telogen phase, but the
telogen count may still be within the range of normal. Inflammation is
uncommon, and fibrous streamers such as those found in androgenetic alopecia are
absent. The follicles are not as long or as wide as normal, but miniaturization
such as that seen in androgenetic alopecia is absent. All these subtle findings
would be uniform over the scalp surface.
The following combination of histological findings (based on 4-mm punch
biopsy specimens) is typical of senescent alopecia (Figures 7.1 and 7.2):
(1) A slight decrease in the total number of hairs (2035 as opposed to the normal
3045)
(2) Numbers of telogen hairs within the range of normal (less than 15%)
(3) A normal percentage of terminal hairs, with terminal hairs outnumbering
vellus and indeterminate hairs by at least 2:1
Figure 7.1 The crown in senescent alopecia. The specimen is from an elderly patient with
mild, diffuse thinning of hair who complained of gradual hair loss over a period of several
years. The two specimens (here and in Figure 7.2) closely resemble each other and are
within the range of normal for number, size and follicular phase of hairs. Original
magnification 40
Figure 7.2 The occiput in the same patient as in Figure 7.1. Original magnification 40
67
SUMMARY
Clinical correlation: an elderly person who admits to very gradual thinning of
the hair; hair density appears normal (for age) or diffusely thinned over the entire
scalp.
Histological findings:
Hordinsky M, Sawaya M, Roberts JL Hair loss and hirsutism in the elderly. Clin Geriatr
Med 2002; 18:12133
Kligman AM. The comparative histopathology of male-pattern baldness and senescent
baldness. Clin Dermatol 1988;6:10818
Sperling LC, Lupton GR The histopathology of non-scarring alopecia. J Cutan Pathol
1995; 22:97114
CHAPTER 8
Androgenetic alopecia
ANDROGENETIC ALOPECIA 69
Figure 8.1 Typical androgenetic alopecia in a young woman. Thinning is most prominent
over the crown
Figure 8.2 The same patient as in Figure 8.1. There is relative sparing of the occiput
normal ratio of terminal: vellus hairs should be at least 2:1, but specimens
diagnostic of androgenetic alopecia show a reduction in this ratio. In advanced
cases, the number of vellus and indeterminate hairs will actually surpass the
number of terminal hairs (Figures 8.10 and 8.11). Early in the course of balding,
the total number of follicles present remains normal. Also, sebaceous glands
persist even when the hairs have greatly miniaturized (Figure 8.12). However, in
very long-standing balding, there is an actual decrease in follicular density as
well as follicular size. Therefore, androgenetic alopecia shows a biphasic pattern
of hair loss and may eventually resemble cicatricial alopecia.
As hairs miniaturize, the anagen phase becomes shorter. Therefore, the balding
scalp shows an increase in the telogen count, since miniaturized hairs spend a
greater proportion of each hair cycle in the telogen phase. In well-established
androgenetic alopecia, the majority of telogen follicles will be miniaturized
follicles (Figure 8.13). Successful treatment with minoxidil or finasteride will
increase the size of follicles and reduce the telogen count, presumably by
lengthening the duration of anagen.
ANDROGENETIC ALOPECIA 71
Figure 8.3 Section (involved vertex) taken at the level of the dermal/fat junction. In this
woman with early androgenetic alopecia, the number of follicles in the vertex specimen is
similar to the number in the occipital specimen (Figure 8.4). However, follicles in the
vertex specimen show considerable variation in diameter, and many are quite small.
Original magnification 40
Figure 8.4 The same patient as in Figure 8.3, with this section taken from the normal
occiput. Original magnification 40
SUMMARY
Clinical correlation: symmetric thinning, predominantly affecting crown, vertex
and frontal regions, with relative sparing of the occiput; no evidence of scarring.
Family history of balding is usually elicited.
Histological findings (Figures 8.14 and 8.15):
ANDROGENETIC ALOPECIA 73
Figure 8.5 Involved vertex. The same specimen as in Figure 8.3, but here sectioned at the
level of the mid- to upper dermis. The marked variation in hair size in the vertex specimen
is evident However, the total number of hairs in both vertex and occipital (Figure 8.6)
specimens appears normal. Original magnification 40
No significant inflammation
BIBLIOGRAPHY
Kligman AM. The comparative histopathology of male-pattern baldness and senescent
baldness. Clin Dermotol 1988; 6:10818
Lattanand A, Johnson WC. Male pattern alopecia: a histopathologic and histochemical
study. J Cutan Pathol 1975; 2: 5870
Sperling LC, Lupton GR The histopathology of non-scarring alopecia. J Cutan Pathol
1995; 22:97114
Van Neste D, Fuh V, Sanchez-Pedreno P, et al. Finasteride increases anagen hair in men
with androgenetic alopecia. Br J Dermatol 2000; 143:80410
Whiting DA,Waldstreicher J,Sanchez M, Kaufman KD. Measuring reversal of hair
miniaturization in androgenetic alopecia by follicular counts in horizontal sections of
Figure 8.6 Normal occiput. The same specimen as in Figure 8.4, but here sectioned at the
level of the mid- to upper dermis. Original magnification 40
serial scalp biopsies: results of finasteride I mg treatment of men and
postmenopausal women. J Invest Dermatol Symp Proc 1999; 4:2824
Whiting DA. Diagnostic and predictive value of horizontal sections of scalp biopsy
specimens in male-pattern androgenetic alopecia. J Am Acad Dermatol 1993; 28:
75563
ANDROGENETIC ALOPECIA 75
Figure 8.8 Old streamer, in the same woman as in Figure 8.7. Original magnification
400
Figure 8.9 Relatively new stela, in a 16-year-old girl with androgenetic alopecia. Such
streamers, found in more recently miniaturized hairs, show more prominent vascularity.
Original magnification 400
Figure 8.10 Involved vertex. The same specimen as in Figure 8.3, sectioned at the level
of the mid- to upper dermis. When multiple follicular units from the balding scalp are
examined, most will show that vellus hairs equal or even outnumber terminal hairs.
Original magnification 200
ANDROGENETIC ALOPECIA 77
Figure 8.11 Normal occiput. The same specimen as in Figure 8.5, sectioned at the level
of the mid- to upper dermis. Here, when multiple follicular units from the normal scalp
are examined, terminal hairs outnumber vellus hairs. Original magnification 200
Figure 8.12 A follicular unit in which all hair shafts have miniaturized. Even so, the
sebaceous glands remain intact. Original magnification 200
Figure 8.13 Involved crown of a woman with androgenetic alopecia. The percentage of
telogen hairs is increased, but the majority of telogen hairs are vellus or indeterminate
hairs. Original magnification 200
ANDROGENETIC ALOPECIA 79
Figure 8.14 A 16-year-old girl with marked androgenetic alopecia. All the typical
histological features are present, including: normal number of follicles; variation in
follicular size; vellus and indeterminate follicles outnumbering terminal follicles;
increased percentage of catagen/telogen follicles; and no significant inflammation.
Original magnification 40
Figure 8.15 The same specimen as in Figure 8.14. Original magnification 200
CHAPTER 9
Telogen effluvium
A telogen effluvium occurs when abnormally large numbers of anagen hairs from
all areas of the scalp enter the telogen phase. This may be caused by some sort of
endogenous stress to the follicles, such as a metabolic disturbance, nutritional
deficiency, or serious systemic illness. Other cases of telogen effluvium are
physiological, and not indicative of disease. The many possible causes are
listed in Table 9.1. In response to the causative factor, many hairs prematurely
enter the catagen phase. This is a committed step for follicles; having entered
catagen they must proceed through the telogen phase and shedding before a new
anagen hair can regrow.
Telogen effluvium is probably the most common form of hair loss associated
with systemic diseases, especially chronic and debilitating conditions. However,
only the most dramatic cases of telogen effluvium, resulting in more than 25%
hair loss, are likely to come to clinical attention. Most drugs that have been
associated with hair loss, with the exception of anticancer medicines and other
toxins, cause hair loss by way of a telogen effluvium.
Physiological forms of telogen effluvium include postpartum and neonatal
hair loss. In postpartum telogen effluvium, numerous hair follicles are artificially
maintained in the anagen state under the influence of gestational hormones. This
is reflected in the lower telogen counts that occur towards the end of pregnancy.
After parturition, numerous follicles suddenly enter the catagen/telogen phase,
and shedding of hairs begins about 3 months later.
Telogen effluvium of the newborn represents the nearly universal shedding of
scalp hair during the first
Table 9.1 Causes of telogen effluvium
Physiological (not pathological)
Physiological effluvium of the newborn
Postpartum
Injury or stress (pathological)
Post-febrile (extremely high fevers, e.g. malaria)
Severe infection
Severe chronic illness
Severe, prolonged psychological stress
6 months of life. This may occur rapidly, resulting in obvious alopecia, or may
proceed slowly and imperceptibly. In either case, large numbers of anagen hairs
enter telogen within a period of months.
Many adult women suffer from a chronic telogen effluvium with no definable
precipitating event. This has been termed chronic telogen effluvium, and is a
diagnosis of exclusion. Many or most of these women have self-limited disease,
although the condition may last for several years before spontaneous resolution.
The early stage of androgenetic alopecia has features of a chronic, localized
form of telogen effluvium. The vertex and frontal hairs of the balding scalp
experience a marked reduction in the length of anagen. A much higher
proportion of hairs are thus entering telogen at any given time. Hair shedding is
only obvious during the early stages of the balding process, when large, terminal
hairs are being shed. Once hairs have miniaturized, the shedding of vellus
telogen hairs is not apparent.
Patients with acute forms of telogen effluvium notice hair loss about 3 or 4
months after the precipitating event. This corresponds to the time it takes for a
hair to move through catagen and the early stages of telogen. Scalp hair density
may appear normal, despite the patients complaint of profuse hair loss. If alopecia
is clinically obvious, the loss appears diffuse, affecting all parts of the scalp
(Figure 9.1). A gentle hair pull will yield several hairs with the depigmented,
cornified, clubbed morphology of telogen hair roots. A forcible hair pluck will
produce a mixture of normal anagen and telogen hairs, as well as an occasional
catagen hair (Figure 9.2). The percentage of telogen hairs will be increased to
more than 20%, a criterion without which the diagnosis of telogen effluvium
cannot be established with certainty. In the typical case of telogen effluvium, the
telogen count does not exceed 50%. However, exceptions to this rule have been
reported, and counts can reach 80%. Figures exceeding 80% are inconsistent
with a simple case of telogen effluvium.
HISTOLOGICAL FINDINGS
Just as in androgenetic alopecia, the histological diagnosis of telogen effluvium
depends more on quantitative than qualitative features. The only abnormality in
a pure case of telogen effluvium is an increase in the percentage of terminal
TELOGEN EFFLUVIUM 83
Figure 9.1 This patient with newly diagnosed hypothyroidism demonstrates the typical
pattern of telogen effluviumthinning of hair over the entire scalp, including the
occipital and parietal regions
telogen follicles. Therefore, the total number of hairs in the specimen will be
normal, but there will appear to be a reduced number of terminal hairs when
counted at the dermal-fat junction. Fibrous streamers (stelae) replace the
missing terminal hairs at this level (Figures 9.3 and 9.4). These streamers lie
beneath the bulbs of the terminal telogen hairs, which are found in the middermis. Once counted, the telogen hairs are increased in number, and the telogen
count (number of terminal telogen hairs divided by the total number of terminal
hairs) will be greater than 20%. However, a lower number does not exclude the
diagnosis of telogen effluvium. If a patients normal telogen count happens to be
5%, a telogen count of 15% would be clearly abnormal for that patient.
Unfortunately, we do not know the baseline telogen counts for individuals, so
numbers less than 20% must be regarded as equivocal. Values of 1520% are
often found in cases of chronic, low-grade telogen effluvium. Even lower
numbers can be consistent with this diagnosis.
Figure 9.2 Portion of a forcible hair pluck (trichogram) obtained from the scalp of a
young woman with a telogen effluvium secondary to systemic lupus erythematosus. There
is a mixture of normal-appearing anagen and telogen bulbs, but the telogen bulbs are overrepresented. When all hairs were counted, the telogen count was 35%. Original
magnification 40
Figure 9.3 Telogen effluvium secondary to retinoid therapy. This specimen, sectioned at
the level of the dermal-fat junction, demonstrates a reduced number of terminal anagen
hairs. However, the terminal anagen hairs present are roughly similar in diameter. Several
catagen/telogen hairs are seen, as well as a few stelae (underlying additional catagen/
telogen hairs). After all follicles were counted, the total number was normal but the
percentage of telogen hairs was elevated at 40%. Original magnification 40
TELOGEN EFFLUVIUM 85
Figure 9.4 The same specimen as in Figure 9.3 (T, telogen follicle). Original
magnification 100
CHAPTER 10
Trichotillomania
Figure 10.1 Typical lesion of trichotillomania.The site chosen for the biopsy samples is
evident
Frequently, chunks of pigmented hair matrix or cortex cells are torn from their
moorings during the plucking process, and come to rest in superficial portions of
the follicles (Figure 10.7). These cells then shrink to form a dark black
homogeneous clump called a pigment cast (Figure 10.8). Pigment casts are
simply the byproduct of fragmented, ectopic matrix or cortical epithelium.
If the hair matrix and suprabulbar epithelium is injured, but not severely
disrupted, the follicle may remain in the anagen phase, producing a hair shaft.
However, the shaft that is formed may be distorted in shape, smaller than normal
and incompletely cornified. This is termed trichomalacia (Figures 10.910.11).
Although trichomalacia is very characteristic of trichotillomania, it is not found
exclusively in this condition (refer to the section on alopecia areata, Chapter 14).
Pulling or plucking does not incite inflammation but may cause some
hemorrhage within the lower portion of the follicle (Figure 10.12). Even very
dramatic cases of trichotillomania are remarkably free of an inflammatory
TRICHOTILLOMANIA 89
Figure 10.3 A similar follicle (on the left) to that in Figure 10.2. On the right is a normal
follicle for comparison. Original magnification 200
Figure 10.4 Catagen hairs are frequently found in trichotillomania. Two catagen hairs are
present in this field. The follicle on the right is sectioned through the newly forming
club. Original magnification 400
infiltrate. Very rarely, a few eosinophils are found surrounding the lower portion
of a badly traumatized follicle.
SUMMARY
Clinical correlation: the patient is often a child or teenager, and a history of
emotional stress at home, school or work may be elicited. The involved areas are
irregularly shaped, sharply marginated patches with some retained short hairs of
various lengths.
Histological findings:
Figure 10.5 Increased numbers of telogen hairs are seen in this specimen from a patient with
trichotillomania. Original magnification 400
Figure 10.6 Increased numbers of telogen hairs (or underlying stelae) are seen in this
specimen from a patient with trichotillomania. The histological differential diagnosis at
this level would include telogen effluvium. Original magnification 40
TRICHOTILLOMANIA 91
Figure 10.7 A clump of non-cornified cortex cells has been stranded at an ectopic level
during the plucking process. Eventually, such cells shrink and cornify to form pigment
casts. Original magnification 400
Figure 10.8 A pigment cast within the center of a telogen follicle. Original magnification
400
BIBLIOGRAPHY
Bergfeld W, Mulinari-Brenner F, McCarron K, Embi C. The combined utilization of
clinical and histological findings in the diagnosis of trichotillomania. J Cutan Pothol
2002; 29:20714
Muller SA. Trichotillomania: a histopathologic study in sixty-six patients. J Am Acad
Dermotol 1990; 23:5662
Sperling LC, Lupton GR The histopathology of non-scarring alopecia. J Cutan Pothol
1995; 22:971 14
Walsh KH, McDougle CJ. Trichotillomania. Presentation, etiology, diagnosis and
therapy. Am J Clin Dermatol 200l; 2:32733
Whiting D. Dermatopathology of common hair problems. J Cutan Med Surg 1999; 3
(Suppl 3):S313
Figure 10.9 Trichomalacia. Two black pigment casts and two trichomalacic shafts can be
seen. Original magnification 400
TRICHOTILLOMANIA 93
Figure 10.11 Trichomalacia. Another example with a single trichomalacic shaft. Original
magnification 400
Figure 10.12 Extravasated red blood cells in the bulb of a catagen hair. Original
magnification 200
CHAPTER 11
Traction alopecia
TRACTION ALOPECIA 95
Figure 11.1 Thinning of hair around the margins of the scalp in a girl with a history of
tightly braided hair
Figure 11.2 Burnt out or end-stage traction alopecia in a 30-yeaf-old woman with
permanent hair loss, confirmed by biopsy
Figure 11.3 Mild traction alopecia in an adult woman. The number of terminal anagen
hairs is reduced in the alopecic zone (seen here) as compared with normal-appearing
perilesional skin (see Figure 11.4). Original magnification 40
Figure 11.4 Normal-appearing perilesional skin contrasts with the lesional specimen
shown in Figure 11.3. Original magnification 40
SUMMARY
Clinical correlation:
TRACTION ALOPECIA 97
Figure 11.5 Low-power view of a specimen from a woman with end-stage traction
alopecia. The reduction in the number of terminal hairs is striking, but sebaceous glands
are preserved. Original magnification 40
Figure I 1.6 Another low-power view of a specimen from a second woman with endstage traction alopecia. Original magnification 40
Figure 11.7 Low-power view of a specimen from a third woman with end-stage traction
alopecia. Original magnification 40
Figure 11.8 Specimen from a woman with end-stage traction alopecia. Terminal hairs are
markedly reduced from normal, but normal numbers of vellus hairs persist. Original
magnification 100
Figure 11.9 Specimen from a different woman than in Figure 11.8, also showing endstage traction alopecia. Original magnification 100
TRACTION ALOPECIA 99
Figure 11.10 Old stelae in a specimen from a patient with end-stage traction alopecia.
When sectioned transversely, these stelae appear as roughly oval condensations of
connective tissue containing a few small vascular spaces. Original magnification 200
Figure 11.11 Old stela in a specimen from another patient with end-stage traction
alopecia. Original magnification 400
Sperling LC, Lupton GR The histopathology of non-scarring alopecia. J Cutan Pathol
1995;22:97114
SteckWD. Telogen effluvium: a clinically useful concept, with traction alopecia as an
example. Cutis 1978; 21:5438
Trueb RM. Chignon alopecia: a distinctive type of nonmarginal traction alopecia. Cutis
1995; 55:1789
Figure 11.12 Columns of connective tissue marking the sites of former follicles are quite
obvious in this case of traction alopecia. In some cases, each column may represent the
former site of an entire follicular unit. Original magnification 100
CHAPTER 12
Postoperative (pressure-induced) alopecia
This form of hair loss is seen most commonly in patients who have undergone
lengthy surgical procedures, and had one portion of their scalp (usually the
occiput) in prolonged contact with the operating table. Postoperative alopecia can
occur at any age and is often associated with gynecological and open-heart
procedures requiring tracheal intubation. Less commonly, the condition is found
in patients who sustain blunt trauma to the scalp. Postoperative alopecia typically
presents as a solitary, roughly oval patch on the upper occiput (Figure 12.1).
Early in the course of the condition, erythema and induration are found in the
central portion of the lesion. Nearly total hair loss with fairly sharp demarcation
from the surrounding scalp is found just a few weeks after the initial trauma.
Usually complete hair regrowth occurs, although several cases of permanent (i.e.
cicatricial) hair loss have been reported.
HISTOLOGICAL FINDINGS
The histological findings in postoperative alopecia change as the lesion evolves.
Early in the course of the disease, before hair loss is complete, vascular
thrombosis, inflammation and destruction may be seen in the dermis. Alopecia
develops up to 28 days following the surgical procedure. In the typical case of
postoperative alopecia, nearly all terminal follicles will be in the catagen or telogen
phases (Figures 12.2 and 12.3). This synchronized conversion of most or all
terminal hairs to the catagen/telogen phase is highly characteristic of
postoperative alopecia. Trichomalacia may be present, but not the distorted or
incomplete follicular anatomy sometimes found in trichotillomania
(Figure 12.4). Pigment casts are also commonly found (Figure 12.5). Melanin
pigment is usually found in the collapsed root sheaths below catagen/telogen
follicles.
Variable degrees of dermal fibrosis and chronic inflammation are present in
the papillary and upper reticular dermis. Focal vascular and tissue necrosis may
be present along with an associated chronic inflammatory infiltrate. Fat necrosis
is often found, associated with an infiltrate of foamy macrophages and
mononuclear cells (Figure 12.6). Inflammation is mild relative to the degree of
Figure 12.1 Postoperative alopecia. A large, nearly hairless, occipital patch was noted 3
weeks after a prolonged surgical procedure
Figure 12.2 Postoperative alopecia. Almost all follicles are in the catagen/telogen phase.
Original magnification 40
apparent tissue damage. The inflammation does not seem to be centered around
hair follicles, but is usually associated with foci of vascular and tissue necrosis.
SUMMARY
Clinical correlation: a patch of occipital hair loss occurring within a few weeks
of a prolonged surgical procedure requiring general anesthesia.
Histological findings:
Figure 12.3 Postoperative alopecia, as in Figure 12.2. Two catagen hairs can be seen.
Original magnification 200
Figure 12.5 Pigment cast in a patient with postoperative alopecia. Original magnification
400
BIBLIOGRAPHY
Boyer JD,Vidmar DA. Postoperative alopecia: a case report and literature review. Cutis
1994; 54:32l-2
Hanly AJ,Jorda M, Badiavas E,Valencia I, Elgart GW Postoperative pressure-induced
alopecia: report of a case and discussion of the role of apoptosis in non-scarring
alopecia. J Cutan Pathol 1999; 26:35761
Figure 12.6 Fat necrosis with reactive granulomatous inflammation in a patient with
postoperative alopecia. Original magnification 400
Patel KD, Henschel EO. Postoperative alopecia. Anesth Analg 1980; 59:31113
Sperling LC, Lupton GP The histopathology of non-scarring alopecia. J Cutan Pathol
1995; 22:97114
Wiles JC, Hansen RC. Postoperative (pressure) alopecia. J Am Acad Dermatol 1985; 12:
1958
CHAPTER 13
Temporal triangular alopecia
Figure 13.1 Temporal triangular alopecia. This unilateral lesion was first noted when the
patient was 3 years old. The condition may also be congenital and/or bilateral
Figure 13.2 Temporal triangular alopecia. A section through the deep dermis reveals very
few follicles. Original magnification 40
BIBLIOGRAPHY
Armstrong DK, Burrows D. Congenital triangular alopecia. Pediatr Dermatol 1996; 13:
3946
Bargman H. Congenital temporal triangular alopecia. Can Med Assoc J 1984; 15:12534
Bargman H. Congenital triangular alopecia. J Am Acad Dermatol 1988; 18:390
Figure 13.3 A section through the upper dermis (compare with Figure 13.2) in temporal
triangular alopecia demonstrates a normal number of follicles, almost all of which are
very small. Original magnification 40
Figure 13.4 A vertical specimen from a patient with temporal triangular alopecia reveals
a few vellus follicles. It is impossible to quantify the total number and average size of
follicles using vertical sections. Original magnification 100
Feuerman EJ. Congenital temporal triangular alopecia. Cutis 1981; 28:1967
Garcia-Hernandez MJ, Rodriguez-Pichardo A, Camacho F. Congenital triangular alopecia
(Brauer nevus). Pediatr Dermatol 1995; 12:3013
Kenner JR,Sperling LC. Pathological case of the month. Temporal triangular alopecia and
aplasia cutis congenita. Arch Pediatr AdolescMed 1998; 152:12412
Kim HJ, Park KB,Yang JM, Park SH, Lee ES. Congenital triangular alopecia in
phakomatosis pigmentovascularis: report of 3 cases. Acta Derm Venereol 2000;80:
21516
Kubba R, Rook A. Congenital triangular alopecia. Br J Dermatol 1976; 95:6579
Ruggieri M, Rizzo R, Pavone P, Baieli S, Sorge G, Happle R. Temporal triangular
alopecia in association with mental retardation and epilepsy in a mother and
daughter. Arch Dermatol 2000; 136:4267
Sperling LC, Lupton GP The histopathology of non-scarring alopecia. J Cutan Pothol
1995;22:97114
Tosti A. Congenital triangular alopecia. Report of fourteen cases. J Am Acad Dermatol
1987; 16:991 -3
Trakimas CA, Sperling LC. Temporal triangular alopecia acquired in adulthood. J Am
Acad Dermatol 1999; 40:8424
Figure 13.5 The small size of hairs in lesional skin from a patient with temporal
triangular alopecia is easily appreciated when compared to hair diameters in normal,
perilesional scalp (see Figure 13.6). Original magnification 100
Figure 13.6 A specimen showing normal, perilesional scalp in a patient with temporal
triangular alopecia. Original magnification 100
Figure 13.7 Temporal triangular alopecia. The dermis below the miniaturized follicles
appears normal, and stelae are not seen. Original magnification 100
Trakimas C, Sperling LC, Skelton HG 3rd, Smith KJ, Buker JL Clinical and histologic
findings in temporal triangular alopecia. J Am Acad Dermatol 1994; 31:2059
CHAPTER 14
Alopecia areata
Figure 14.1 Alopecia areata affecting the beard area. This patient had no scalp hair loss
disease with the passage of time. Different stages of disease may be present at
the same time at different sites on the same scalp. In any given patient, separate
lesions of alopecia areata may begin, evolve, remit and recur independently of
one another.
The acute stage is seen in rapidly progressive disease or disease of recent onset,
as is found in evolving alopecia totalis or at the advancing margin of an
enlarging bald spot. The total number of follicles appears normal. Several
affected hairs are still terminal anagen follicles, with bulbs in the fat or deep
Figure 14.4 Alopecia totalis in evolution. This patient had rapid, diffuse hair loss
progressing over a period of just a few months
Figure 14.5 Patch of alopecia areata with several exclamation mark hairs
Figure 14.6 Exclamation mark hair. This hair was easily pulled from the scalp. The
wider, distal end (right side) shows a fracture, and the proximal end is narrow and
hypopigmented. Most exclamation mark hairs are in the telogen phase
Figure 14.7 Tapered,pencil point hair gently extracted from a patient with rapidly
progressive alopecia areata
Figure 14.8 Alopecia totalis in evolution, with early sparing of hypopigmented hairs. This
patient had black, but graying hair until his hair loss began. The gray hairs were initially
spared, but eventually they too were shed
Figure 14.9 Acute alopecia areata. The number and size of hairs appears normal, but
there is an increase in the number of catagen/telogen hairs. Original magnification 40
only a minority of cases. Nuclear pyknosis and cell death (apoptosis) occurs not
only in matrix keratinocytes, but also in outer root sheath cells and bulbar
melanocytes. Amorphous clumps of pigment (pigment casts) are occasionally
found within the follicular epithelium as a byproduct of hair matrix degeneration
(Figure 14.21).
Figure 14.10 Acute alopecia areata, higher power image of section shown in
Figure 14.9. Original magnification 100
Figure 14.11 Acute alopecia areata. At this level,the number and size of hairs appears
roughly normal, but the presence of several slightly inflamed stelae (arrows) gives a clue
to the diagnosis. Original magnification 40
Figure 14.12 Acute alopecia areata, with several slightly inflamed stelae. Original
magnification 200
Figure 14.13 CD3+T cells (all T lymphocytes stain brown) surround and invade the
epithelium of an anagen hair. Varying proportions of CD4+ and CD8+T cells may be
present in such an infiltrate. Original magnification 400
Figure 14.14 Eosinophils join the lymphocytic infiltrate surrounding the bulb of this
catagen hair. Original magnification 400
Figure 14.15 Eosinophils join the lymphocytic infiltrate surrounding the bulb of this anagen
hair. Original magnification 1000
Figure 14.16 The hair matrix of this inflamed bulb appears blurred and disorganized.
Original magnification 400
Figure 14.17 Despite the scanty peribulbar infiltrate, the hair matrix appears blurred and
somewhat disorganized. Original magnification 400
anagen arrest (or anagen effluvium) has been applied to those forms of alopecia
characterized by the rapid tapering and shedding of large numbers of anagen
hairs. Anagen arrest is characteristic of chemotherapy-induced alopecia, but the
initial stages of alopecia areata (when terminal hairs are still present) also have
features of an anagen arrest.
Figure 14.18 Suprabulbar zone. The outer root sheath of this inflamed follicle (see also
Figure 14.19) appears blurred and somewhat disorganized. Some lymphocytes have
invaded the outer root sheath. Original magnification 400
Figure 14.19 Another example at the suprabulbar zone. Original magnification 400
Figure 14.20 A group of necrotic matrix cells lies just above the upper pole of the hair
papilla. Original magnification 400
However, after producing a dystrophic (or no) shaft for a period of time, these
anagen hairs eventually enter the catagen/telogen phase.
After remaining in the telogen phase for a period of time (about 100 days in a
normal follicle; an unknown length of time in alopecia areata), hair follicles reenter the anagen phase. Unless the disease has spontaneously subsided, an
Figure 14.21 Clumps of pigment are seen within the epithelium of this catagen hair.
Original magnification 200
inflammatory infiltrate again confronts the newly forming anagen hairs. This
results in a repetition of the pathological process: peribulbar inflammation,
disturbance of anagen hair growth and precipitation into the catagen/telogen
phase. As this process repeats itself over and over, the duration of anagen
becomes briefer, the anagen hairs miniaturize, and an increasingly large
percentage of hairs will be found in the catagen/telogen phase, sometimes
approaching 100% of the total (Figure 14.28).
The third histological stage, which here is referred to as chronic, is found in
stable, long-standing bald patches and in well-established alopecia totalis or
universalis. Terminal anagen hairs, with or without surrounding mononuclear
infiltrate, are rare. Thus, the most familiar histological finding of alopecia areata
may be absent. In addition, all the follicles may become miniaturized, but the
total number of follicles remains normal (Figures 14.29 and 14.30). This remains
true for years or even decades, but eventually follicular dropout may occur (an
example of the biphasic pattern of permanent alopecia; see Figure 4.1). When
follicles are miniaturized, they are often missed on routine vertical sectioning.
Transverse sections are required to examine and count all follicles.
The miniaturized anagen follicles found in chronic disease are situated in the
mid- to lower dermis, usually slightly deeper than normal vellus hairs
Figure 14.22 Inflammation predominantly surrounds the bulbs of anagen hairs (e.g. the
follicle at lower right). Late catagen/telogen follicles (e.g. upper left) are relatively
spared. Original magnification 200
Figure 14.23 Residual inflammation surrounds the bulb of an early catagen hair. Original
magnification 400
Figure 14.24 Residual inflammation surrounds the suprabulbar zone of an early catagen
hair. Original magnification 400
Figure 14.25 The stelae below catagen/telogen hairs may also show residual
inflammation. Original magnification 400
shows features of a catagen hair. Conversely, the bulb may show features typical
of a catagen hair while the suprabulbar zone possesses an anagen-like inner root
sheath with trichohyaline granules (Figures 14.3814.41). Nanogen hairs with
anagenlike bulbs may have some matrix cells in mitosis while others are
undergoing apoptosis (Figures 14.42 and 14.43). Thus, features of active growth
(mitotic cells) and involution (apoptotic cells) are seen simultaneously. The
Figure 14.26 This terminal anagen hair is still producing a hair shaft, but it is only a few
cells in width. Once the inner root sheath has desquamated, the shaft easily fractures at
this constriction to form a pencil point hair. Original magnification 400
Figure 14.27 This anagen hair has produced a cornified inner root sheath, but no cortex/
shaft formation has occurred. Original magnification 1000
Figure 14.28 The percentage of telogen hairs in subacute disease can approach 100%. The
remaining anagen hairs have begun to miniaturize. Original magnification 100
Figure 14.29 Alopecia areata,chronic stage. The majority of the follicles are in the
telogen phase. Only a few terminal anagen hairs are present, and peribulbar inflammation
may be difficult or impossible to identify. Original magnification 40
Figure 14.30 Alopecia areata, chronic stage. See Figure 14.29. Original
magnification100
Figure 14.31 The bulb of this miniaturized anagen hair is located in the mid-dermis.
Original magnification 100
Figure 14.32 Nanogen hair. The bulbar epithelium of this inflamed hair is only a few
cells in thickness,and is thin relative to the diameter of the papilla. Original magnification
400
Figure 14.33 Nanogen hair. This transverse section at the level of the bulb shows the
features of a typical anagen-like nanogen hair. The matrix is thin and small relative to
the papilla. Original magnification 1000
BIBLIOGRAPHY
El Darouti M, Marzouk SA, Sharawi E. Eosinophils in fibrous tracts and near hair bulbs: a
helpful diagnostic feature of alopecia areata. J Am Acad Dermatol 2000; 42:3057
Elston DM, McCollough ML, Bergfeld WF, Liranzo MO, Heibel M. Eosinophils in
fibrous tracts and near hair bulbs: a helpful diagnostic feature of alopecia areata. J
Am Acad Dermatol 1997; 37:1016
Freyschmidt-Paul P, Hamm H, Happle R, Hoffmann R. Pronounced perifollicular
lymphocytic infiltrates in alopecia areata are associated with poor treatment response
to diphencyprone. Eur J Dermatol 1999; 9:11114
Ghersetich I, Campanile G, Lotti T. Alopecia areata: immunohistochemistry and
ultrastructure of infiltrate and identification of adhesion molecule receptors. Int J
Dermatol 1996; 35:2833.
Figure 14.34 Nanogen hair. Transverse section at the level of the suprabulbar zone. There
is no central shaft formation. Original magnification 1000
Kim lH, Jo HY, Cho CG, Choi HC, Oh CH. Quantitative image analysis of hair follicles
in alopecia areata. Acta Dermatol Venereol 1999; 79:21416
Nutbrown M, MacDonald Hull SP, Baker TG, Cunliffe WJ, Randall VA. Ultrastructural
abnormalities in the dermal papillae of both lesional and clinically normal follicles
from alopecia areata scalps. Br J Dermatol 1996; 135:20410
Tobin SJ. Morphological analysis of hair follicles in alopecia areata. Microsc Res Tech
1997; 38:44351
Whiting DA. Histopathology of alopecia areata in horizontal sections of scalp biopsies. J
Invest Dermatol 1995; 104 (5 Suppl):26S-27S
Whiting DA. The histopathology of alopecia areata: a new look. Poster presentation at the
4th International Research Workshop on Alopecia Areata, November 2002.
Washington DC,2003; in press
Figure 14.35 Nanogen hair. Transverse section at a higher level of the suprabulbar zone.
The inner root sheath has cornified, but there is no shaft. (Same follicle as shown in
Figures 14.33 and 14.34.) Original magnification 1000
Figure 14.36 A vertically sectioned anagen-like nanogen hair corresponds to the type of
hair shown in Figures 14.3314.35. The extremely small size of such a hair is best
appreciated by comparing it to the bulb of a normal anagen hair (Figure 14.37),
photographed at the same magnification. Original magnification 400
Figure 14.38 This transversely sectioned nanogen hair has the bulb of a catagen hair.
Original magnification 400
Figure 14.39 Catagen-like suprabulbar zone of the same nanogen hair shown in
Figure 14.38. Original magnification 400
Figure 14.40 More superficial level of the same nanogen hair seen in Figures 14.38 and
14.39, but here showing the anagen-like features of inner root sheath and cortex
formation. Original magnification 400
Figure 14.41 More superficial level of the nanogen hair shown in Figure 14.40. Original
magnification 400
Figure 14.42 This greatly magnified anagen-like nanogen hair has a matrix that is less
basophilic than normal, yet it produces inner root sheaths (found on more superficial
sections). In addition, both mitotic and apoptotic cells are found. Original magnification
1000
Figure 14.43 Another anagen-like nanogen hair bulb, as in Figure 14.42. Original
magnification 1000
Figure 14.44 A catagen-like nanogen hair adjacent to a terminal anagen hair. Note
the dramatic difference in size, and the more superficial location of the nanogen hair.
Original magnification 100
Figure 14.46 A follicle similar to those in Figures 14.44 and 14.45, sectioned transversely
through the bulb. Original magnification 400
Figure 14.49 An unusually dense lymphocytic infiltrate surrounds the bulb of an anagenlike nanogen hair. Original magnification 400
Figure 14.50 An unusually dense lymphocytic infiltrate surrounds the bulb of a catagenlike nanogen hair. Original magnification 400
Figure 14.51 Immunoperoxidase stain for CD3+T cells high-lights the lymphocytes
surrounding the bulb of a nanogen hair. This stain may be helpful in cases where
inflammation is scanty. Original magnification 200
Figure 14.52 In this specimen demonstrating the chronic histological phase of alopecia
areata, the majority of the miniaturized follicles are in the telogen phase, and their bulbs
are not inflamed. Original magnification100
Figure 14.53 Three transversely sectioned stelae (streamers) in a patient with longstanding alopecia totalis. Original magnification 200
CHAPTER 15
Syphilitic alopecia
Syphilis, the great imitator, is capable of causing alopecia with several clinical
and histological patterns. Alopecia found in secondary syphilis may be
associated with other cutaneous lesions, or may be the only external
manifestation of syphilis. The pattern of hair loss can be patchy (moth-eaten),
diffuse or a combination of the two (Figure 15.1).
When papulosquamous lesions of secondary syphilis are found on the scalp in
association with alopecia (symptomatic syphilitic alopecia), the histological
findings are usually those typically associated with lesions of secondary syphilis.
These findings include: a perivascular and perifollicular infiltrate of
lymphocytes, histiocytes and often numerous plasma cells; involvement of both
superficial and deep dermal vascular plexuses; vascular dilatation and
prominent, swollen endothelial cells; and frequent epidermal involvement with
epidermal hyperplasia, spongiosis, infiltration with neutrophils and interface
inflammation.
Hair loss in the absence of papulosquamous lesions of the scalp is termed
essential syphilitic alopecia. The clinical pattern of hair loss may be motheaten or diffuse (as in telogen effluvium). Syphilis is one cause of telogen
effluvium, and in some cases of diffuse syphilitic alopecia the histological
pattern is that of a typical telogen effluvium (described in Chapter 9). However,
more often the pattern is an inflammatory, predominantly peribulbar and
suprabulbar, non-scarring process closely mimicking alopecia areata
(Figures 15.215.6). Catagen/telogen follicles are increased in number, initially
as terminal hairs, but eventually as miniaturized follicles. A mononuclear cell
infiltrate surrounds the inferior segment of the follicle or involves the fibrous
tracts below catagen/telogen hairs (Figure 15.5). A minority of specimens will
show a solitary peribulbar lymphoid aggregate, and in some cases large numbers
of lymphocytes infiltrate the outer root sheath at the isthmus level (Figure 15.6).
In well-established syphilitic alopecia, the majority or all the follicles have
miniaturized (Figures 15.7 and 15.8). The peribulbar infiltrate tends to be rather
scanty, and plasma cells may be difficult to find. Because so many follicles have
miniaturized, finding and counting involved hairs is best achieved with
transverse sectioning. Special stains for spirochetes do not reveal organisms.
Figure 15.1 Syphilitic alopecia that is both diffuse (affecting the entire scalp) and patchy
(affecting some areas more than others). This pattern is typical. Photograph courtesy of
Timothy Berger, MD
Eosinophils in the infiltrate are unusual, but in many cases plasma cells are
present in small numbers.
Syphilitic alopecia and alopecia areata may have the following features in
common:
(1) Sparing of the epidermis
(2) Infiltration of lymphocytes around the lower segment of follicles, in fibrous
tracts, or within the follicular epithelium
(3) Presence of small or abnormal anagen hairs
(4) Markedly increased numbers of catagen/telogen follicles
The presence of plasma cells and lymphocytic infiltration of the outer root sheath
is more likely to be seen in syphilitic alopecia than in alopecia areata.
Serological tests for syphilis and a prompt and complete response to
antitreponemal antibiotics help to confirm the diagnosis.
SUMMARY
Clinical correlation: a diffuse (resembling telogen effluvium) or a diffuse but
moth-eaten alopecia. Other signs and symptoms of syphilis may be present.
Histological findings:
Figure 15.2 Early syphilitic alopecia showing an increased number of catagen hairs.
Original magnification 40. Slide courtesy of James W.Patterson, MD
Figure 15.3 Early syphilitic alopecia. Original magnification 100. Slide courtesy
of James W.Patterson, MD
BIBLIOGRAPHY
Jeerapaet P, Ackerman AB. Histologic patterns of secondary syphilis. Arch Dermatol
1973; 107:3737
Jordaan HF, Louw M. The moth-eaten alopecia of secondary syphilis. A histopathological
study of 12 patients. Am J Dermatopathol 1995; 17:15862
Lee JY, Hsu ML Alopecia syphilitica, a simulator of alopecia areata: histopathology and
differential diagnosis. J Cutan Pothol 1991; 18:8792
Sperling LC, Lupton GR The histopathology of non-scarring alopecia. J Cutan Pathol
1995;22:971 14
Figure 15.4 A relatively mild lymphocytic infiltrate with occasional plasma cells
surrounds the lower portion of a catagen hair in early syphilitic alopecia. Original
magnification 200. Slide courtesy of James W Patterson, MD
Figure 15.5 Residual inflammation is found in the collapsed root sheath (stela) below
a catagen hair. Original magnification 400
Figure 15.6 A collection of lymphocytes has entered the epithelium of this telogen hair.
Original magnification 400
Figure 15.7 Section from a patient with well-established syphilitic alopecia. The follicles
are very small and inflammation is scanty. Original magnification 40
Figure 15.8 Section from a patient with well-established syphilitic alopecia. Original
magnification 100
CHAPTER 16
Non-scarring alopecia from systemic lupus
erythematosus
Hair loss in systemic lupus erythematosus (SLE) can occur in several forms.
Discoid lesions causing scarring alopecia are the most familiar form of alopecia
to both clinician and dermatopathologist, and will be discussed further in
Chapter 21. Patients with SLE can be severely ill for long periods of time, and so
diffuse hair loss can occur in the form of a telogen effluvium, as described in
Chapter 9.
A form of hair loss in SLE that is fairly common but has received little
attention in the literature is patchy, non-scarring alopecia. This form of hair loss
occurs in patients with severe disease, and the underlying diagnosis of SLE has
already been established or is suspected. Patches of partial or total hair loss are
scattered on the scalp (Figure 16.1), and are associated with mild erythema, but
without evidence of scarring (follicular ostia and surface texture appear normal).
The hairs remaining in the balding patches are almost all telogen hairs or
dystrophic pencil-point anagen hairs (Figure 16.2), a finding diagnostic of an
anagen arrest (described in Chapter 14). If the underlying disease is promptly
brought under control, complete hair regrowth occurs.
HISTOLOGICAL FINDINGS
A peribulbar mononuclear cell infiltrate is found around anagen hair bulbs, many
of which become miniaturized with the passage of time (Figures 16.316.7). The in
flammatory infiltrate may be more dense than that found in alopecia areata, but
is not always so. The percentage of catagen and telogen hairs is greatly increased,
and may approach 100% (Figure 16.8). Pigment incontinence and a mild
inflammatory infiltrate are often found in the collapsed root sheaths (stelae)
below telogen hairs.
These histological findings are similar to those found in alopecia areata and
essential syphilitic alopecia, and a diagnosis of lupus erythematosus may not be
possible on histological grounds alone. Clinical findings and serological testing
may be required to differentiate reliably between these three forms of nonscarring, reversible, inflammatory alopecia. However, some additional
histological features may help to distinguish SLE from its histological mimics.
For instance, an increase in dermal mucin is sometimes present (Figure 16.9).
Figure 16.1 Young woman with active systemic lupus erythematosus and patchy, nonscarring hair loss. Note the mild, mottled erythema
Figure 16.2 Forcible hair pluck (trichogram) taken from a markedly thinned patch of
scalp in a patient with active systemic lupus erythematosus. The hairs are all telogen
hairs, because all the anagen hair shafts have been shed. Original magnification 40
Although increased mucin may be visible with routine staining, colloidal iron or
similar stains can accentuate the mucin. Inflammation around eccrine glands and
dermal vasculature is often seen in the alopecia of SLE. When inflammation
involves dermal blood vessels, extravasated red blood cells are sometimes found
(Figure 16.10). If present, an especially dense inflammatory infiltrate supports a
diagnosis of lupus erythematosus (Figure 16.11). Finally, focal areas of vacuolar
basilar degeneration, affecting the infundibula of follicles, may be found in some
cases of SLE alopecia (Figure 16.12).
SUMMARY
Clinical correlation: the patient is usually a young adult with severe disease, and
the underlying diagnosis of SLE has already been established or is suspected.
Patches of partial or total hair loss are scattered on the scalp. Mild erythema of
the scalp may be present.
Figure 16.3 Terminal anagen hairs still predominate in this early lesion of patchy, nonscarring systemic lupus erythematosus. A moderately dense lymphocytic infiltrate
surrounds the lower segments of several anagen hairs, but involves interfollicular blood
vessels as well. Original magnification 100
Figure 16.4 Patchy, non-scarring systemic lupus erythematosus (see Figure 16.3). The
perivascular infiltrate contains some plasma cells. Original magnification 400
Figure 16.9 Even without special stains, interstitial dermal mucin is obvious in this
specimen from a patient with patchy, non-scarring systemic lupus erythematosus
alopecia. Original magnification 200
Figure 16.10 Deep perivascular inflammation with extravasated red blood cells in a
patient with patchy, non-scarring systemic lupus erythematosus alopecia. Original
magnification 400
Figure 16.11 Deep, dense, chronic inflammation in a patient with patchy, nonscarring systemic lupus erythematosus alopecia. Original magnification 200
Figure 16.12 Mild and focal vacuolar interface change affecting the follicular
infundibulum in a patient with patchy, non-scarring systemic lupus erythematosus
alopecia. Original magnification 400
CHAPTER 17
Loose anagen hair syndrome
Although loose anagen hair syndrome (LAHS) was first described in the late
1980s, it is a fairly common form of non-scarring hair loss. Lack of familiarity with
the condition is the major obstacle to establishing the diagnosis. Most patients
are first diagnosed at the age of 25 when they are brought to a physician with
the complaint of thin, uneven hair with an abnormal texture (Figures 17.117.3).
Parents often state that the childs hair wont grow.
Anagen hairs can be easily and painlessly extracted from the scalp by gentle
traction. Extracted hairs show a characteristic ruffling of the hair shaft cuticle
(Figure 17.4). The inner and outer root sheaths are left behind in the scalp when
anagen hairs are extracted. Some authors have found an increased telogen count
(> 20%) in patients with LAHS, but others have found a decreased count (<
5%). This issue will require more careful study in large numbers of patients.
HISTOLOGICAL FINDINGS
There are no reports of inflammation in biopsy specimens from LAHS patients.
The internal root sheath has been reported to show a crumbling degeneration as
well as premature keratinization. Crumbling degeneration may refer to the
numerous fractures that may occur within the cornified inner root sheath during
tissue processing (Figures 17.5 and 17.6). This artifact of processing can also be
seen in normal specimens, but is more common and prominent in LAHS.
Varying degrees of abnormal (i.e., excessive) artifactual separation can be seen
between the hair cuticle and cortex, between the hair cuticle and inner root
sheath cuticle, within the inner root sheath, and between the inner and outer root
sheaths (Figures 17.717.9). This clefting is not specific for LAHS, and similar
changes can occasionally be found in specimens from normal scalps. However,
artifactual clefting appears to be a prominent and consistent feature found in
LAHS.
It must be emphasized that clefting found between the cornified hair shaft and
inner root sheath is an artifact of processing that occurs in almost all normal as well
as abnormal follicles. It is therefore irrelevant and should be ignored.
Occasionally, incomplete follicular anatomy resembling trichotillomania is
found. When this occurs, only the hair shaft is absent, and the residual inner and
Figure 17.1 A 4-year-old boy with loose anagen hair syndrome. The hair is focally
thinned with locks that are uneven in length. Some spangling of the hair is evident, caused
by the irregular grooves and twists in some of the hair shafts
outer root sheaths are collapsed (Figure 17.10). This finding is hardly surprising,
because the shafts can be easily and painlessly extracted in children with LAHS,
and sites of recent depilation may be chosen for biopsy.
Although most hair shafts in LAHS retain the normal oval or circular shape,
some shafts have unusual polygonal shapes (Figures 17.11 and 17.12). This
corresponds to the grooving of hair shafts that can often be found in LAHS. A
few patients with LAHS have more prominent hair shaft grooving, affecting
most hairs. In some specimens from patients with LAHS, an increased telogen
count is found.
SUMMARY
Clinical correlation: child with areas of slightly thinned, somewhat unruly hair.
Parents complain that childs hair wont grow.
Histological findings:
Figure 17.2 A 4-year-old girl with loose anagen hair syndrome. Most reported patients
have been girls with blond hair. The hair is somewhat thinned and has a windblown
appearance
BIBLIOGRAPHY
Baden HP, Kvedar JC, Magro CM. Loose anagen hair as a cause of hereditary hair loss in
children. Arch Dermatol 1992; 128:134953
Boyer JD, Cobb MW, Sperling LC, Rushin JM. Loose anagen hair syndrome mimicking
the uncombable hair syndrome. Cutis 1996; 57:l 1112
Chapman DM, Miller RA. An objective measurement of the anchoring strength of anagen
hair in an adult with the loose anagen hair syndrome. J Cutan Pathol 1996; 23:
28892
LiVW, Baden HP, Kvedar JC. Loose anagen syndrome and loose anagen hair. Dermatol
Clin 1996; 14:74551
Nunez J, Grande K, Hsu S. Alopecia areata with features of loose anagen hair. Pediatr
Dermatol 1999; 16:4602
ODonnell BP, Sperling LC, James WD. Loose anagen hair syndrome. Int J Dermatol
1992; 31:1079
Price VH, Gummer CL Loose anagen syndrome. J Am Acad Dermatol 1989; 20:24956
Pride HB, Tunnessen WW Jr. Picture of the month. Loose anagen syndrome. Arch
Pediatr Adolesc Med 1995; 149: 81920
Figure 17.3 Unruly, twisted hair in a girl with loose anagen hair syndrome (LAHS). This
degree of hair shaft abnormality is exceptional for LAHS. Photograph courtesy of Jeffrey
Miller, MD
Sinclair R, Cargnello J, Chow CW. Loose anagen syndrome. Exp Dermatol 1999; 8:
2978
Tosti A, Piraccini BM. Loose anagen hair syndrome and loose anagen hair. Arch Dermatol
2002; 138:5212
Tosti A, Peluso AM, Misciali C, Venturo N, Patrizi A, Fanti PA. Loose anagen hair. Arch
Dermatol 1997; 133:108993
Figure 17.4 Anagen hair gently extracted from the scalp of a child with loose anagen hair
syndrome. The soft hair matrix and suprabulbar zone is prone to kinking during extraction,
resulting in a hockey stick appearance. There is a ruffling of the hair shaft cuticle where
it separates from the inner root sheath. Original magnification 100
Figure 17.5 Numerous fractures within the inner root sheath are commonly found in loose
anagen hair syndrome. Original magnification 400
Figure 17.6 Inner root sheath in loose anagen hair syndrome, showing numerous
fractures. Original magnification400
Figure 17.7 Several variations of artifactual clefting can be seen in loose anagen hair
syndrome. Here clefting has occurred between the hair cortex and the inner root sheath.
Original magnification 400
Figure 17.8 Artifactual clefting in loose anagen hair syndrome. Here clefts between inner
and outer root sheaths are seen. Original magnification 400
Figure 17.9 A circumferential cleft has occurred within the inner root sheath between
Henles and Huxleys layer. Original magnification 400
Figure 17.10 In this specimen from a child with loose anagen hair syndrome, hair shafts
have been previously extracted from two follicles, and the inner root sheaths have
collapsed inward. Original magnification 200. Slide courtesy of Jeffrey Miller, MD
CHAPTER 18
Central, centrifugal scarring alopecia
The term central, centrifugal scarring alopecia (CCSA) was recently coined to
incorporate several variants of inflammatory, scarring alopecia. The difference
between these variants may be due to racial differences or the well-recognized
variability in immune responses between individuals. The entities grouped under
CCSA include pseudopelade (not Brocqs pseudopelade), the follicular
degeneration syndrome, and folliculitis decalvans. As variants of CCSA, they
have the following features in common:
(1) They are chronic and progressive, with eventual spontaneous burn out
after years or decades;
(2) They are predominantly centered on the crown or vertex;
(3) They progress in a roughly symmetrical fashion, with the most active
disease activity occurring in a peripheral zone of variable width, surrounding
a central, alopecic zone;
(4) They show both clinical and histological evidence of inflammation in the
active, peripheral zone.
Over the years, CCSA has been given several names, because the condition
exhibits a spectrum of inflammation. Patients with highly inflammatory disease,
as evidenced by pustule formation, crusting, intense erythema and bacterial
superinfection, have usually been labeled with folliculitis decalvans. Patients
with more indolent disease, characterized by perifollicular scaling and
occasional papule formation, have been labeled with pseudopelade or the
follicular degeneration syndrome. Most patients described as having
pseudopelade have been Caucasians, and most patients labeled with the
follicular degeneration syndrome have been dark-skinned persons of African
descent (Blacks; African Americans). However, clinically and especially
histologically, the similarity of CCSA between racial groups far exceeds any
differences.
CCSA is the most common form of scarring alopecia in any population that
includes significant numbers of Black patients. Among African Americans,
CCSA is responsible for more cases of scarring alopecia than all other forms
combined. The majority of Black patients with CCSA are women, with a female:
Figure 18.1 Early central, centrifugal scarring alopecia emerges as a zone of partial hair
thinning centered on the crown or vertex. Permanent hair loss is evidenced by the loss of
follicular ostia between remaining follicles
Figure 18.2 Even in fairly advanced central, centrifugal scarring alopecia, evidence of
clinical inflammation such as papules and pustules may be absent,and the patient may be
misdiagnosed as having androgenetic alopecia
male ratio of about 3:1. The average age at presentation is 36 years for women
and 31 years for men, although most patients have had progressive disease for
years or decades before they seek medical attention. The disease invariably
begins and remains most severe on the crown or vertex of the scalp (Figure 18.1),
gradually expanding in a centrifugal fashion. Even when the amount of hair loss
is dramatic, symptoms may be mild or absent. Most patients note only mild,
episodic pruritus or tenderness of involved areas. Virtually all African American
women with CCSA are using or have used chemical hair relaxers for styling
purposes, but few men have used anything except pomades. Patients experience
progression of the disease even after all chemical treatments (if any)
are discontinued. Caustic cosmetics may aggravate the disease or hasten its
progression, but cannot fully explain its pathogenesis.
The crown and/or vertex of the scalp shows a symmetrical zone of partial or
complete alopecia. When inflammation is subtle, CCSA may be incorrectly
diagnosed as androgenetic alopecia (Figure 18.2). The follicular ostia between
remaining hairs are obliterated, and the scalp is smooth and shiny, evidence of
scarring alopecia.
Figure 18.3 In the folliculitis decalvans variant of central, centrifugal scarring alopecia,
numerous papules and pustules surround a zone that is almost completely devoid of hair
Figure 18.4 The sections shown in Figures 18.418.7 are well below the level of the
isthmus and eccrine coils. Here, two follicles are shown that have undergone premature
desquamation of the inner root sheath. Original magnification 400
Figure 18.5 One of four follicles, upper right, has undergone premature desquamation of
the inner root sheath. Original magnification 100
Figure 18.6 The follicle from Figure 18.5 that had undergone premature desquamation of
the inner root sheath. Original magnification 200
As the disease evolves in clinically abnormal scalp skin, involved follicles also
demonstrate some or all of the following histological features: eccentric epithelial
atrophy (thinning) with hair shafts in close proximity to the dermis (Figures 18.8
and 18.9); concentric lamellar fibroplasia (onion skin-like fibrosis) of affected
follicles (Figures 18.10 and 18.11); variably dense lymphocytic perifollicular
inflammation, primarily at the level of the upper isthmus and lower infundibulum
(Figures 18.1218.14); occasional fusion of infundibula (polytrichia;
Figures 18.15 and 18.16); and in advanced lesions, total destruction of the
follicular epithelium with retained hair shaft fragments and granulomatous
inflammation. Inter-face alteration of the follicular epithelium is not found. To
date, the immunofluorescent findings in patients with CCSA have not been
critically studied.
When highly inflammatory disease with pustule formation is present
(folliculitis decalvans), the microscopic findings in pustular lesions include
intrafollicular and perifollicular infiltrates rich in neutrophils as well as
lymphocytes (Figures 18.1718.21).
However, if non-pustular lesions at the active periphery are sampled, or if the
biopsy is performed during a period of suppressive therapy, the histological
Figure 18.7 A normal follicle (lower left) and two that have undergone premature
desquamation of the inner root sheath. Original magnification 100
picture is identical to that of other forms of CCSA (Figure 18.22). If early lesions
are sampled, all the histological changes described for CCSA can be found in
folliculitis decalvans.
The only unique histological characteristic of CCSA is premature
desquamation of the inner root sheath. The remainder of the histological features
can be found in other forms of inflammatory, scarring alopecia, and are therefore
suggestive, but not diagnostic.
SUMMARY
Clinical correlation: an adult, most often a Black woman, with a progressive,
permanent loss of scalp hair starting on the central crown or vertex. A spectrum
of clinical inflammation exists, ranging from minimal to highly inflamed, with
marked erythema, pustules and crusting (folliculitis decalvans).
Histological findings: the earliest finding is premature desquamation of the
inner root sheath. This may be found even when normal-appearing scalp skin or
perilesional skin is sampled. In more advanced lesions, some or all of the
following features are usually seen:
Figure 18.8 Once a follicle has undergone premature desquamation of the inner root
sheath, it is prone to eccentric epithelial atrophy (thinning) with a hair shaft in close
proximity to the dermis. Original magnification 200
Figure 18.9 Two abnormal follicles in different stages of evolution; one follicle (lower
left) shows eccentric epithelial atrophy and the second (lower right) shows total epithelial
destruction with a retained hair shaft. A normal follicle (upper-most) is present for
comparison. Original magnification 200
Sperling L, Skelton H, Smith K, Sau R Friedman K. The follicular degeneration syndrome
in men. Arch Dermatol 1994; 130: 7639
Sperling L, Solomon A, Whiting D. A new look at scarring alopecia. Arch Dermatol 2000;
136:23542
Templeton S, Solomon A. Scarring alopecia: a classification based on microscopic
criteria. J Cutan Pathol 1994;21:97109
Whiting DA. Cicatricial alopecia:clinico-pathological findings and treatment. Clin
Dermatol 2001; 19:21125
Figure 18.10 Vertical section of a follicle showing concentric lamellar fibroplasia (as
well as epithelial atrophy) at the level of the upper isthmus/lower infundibulum. Original
magnification 200
Figure 18.11 Transverse section of a follicle similar to that in Figure 18.10. Original
magnification 200
Figure 18.14 One of the two follicles (left side) has an intact inner root sheath and is not
affected by inflammation or fibroplasia. Original magnification 200
Figure 18.15 The infundibula of several adjacent, inflamed follicles may merge, resulting
in clinical polytrichia. Original magnification 200
Figure 18.16 During the healing process a single, common enlarged infundibulum may
form, resulting in polytrichia. Original magnification 200
Figure 18.17 Biopsy specimen taken from a crusted, pustular lesion in a patient with the
folliculitis decalvans variant of central, centrifugal scarring alopecia. Much of the
follicular epithelium has been damaged or destroyed by a dense inflammatory infiltrate
composed predominantly of neutrophils. (See Figures 18.1818.20.) Original
magnification 40
Figure 18.18 Higher power view of the section shown in Figure 18.17. Original
magnification 200
Figure 18.19 Special stain for bacteria reveals colonies of staphylococci. Original
magnification 400
Figure 18.20 Special stain for fungi reveals clusters of fungal spores typical of
Pityrosporum contained within the infundibulum and overlying scale/crust. The
findings in Figures 18.1718.21 are non-specific end stage changes, and can be
found in any highly inflammatory form of scarring alopecia. Original magnification
400
CHAPTER 19
Lichen planopilaris
Figure 19.1 Most commonly, the scalp lesions of lichen planopilaris are irregularly
shaped and widely scattered over the scalp
Figure 19.2 A confetti-like pattern of numerous small and widely distributed lesions is a
relatively common clinical variant of lichen planopilaris
Figure 19.3 Before the hair is completely destroyed, perifollicular erythema and scaling
indicate the presence of active lichen planopilaris
Figure 19.4 This patient with lichen planopilaris has extensive hair loss centered on the
crown of the scalp, resembling a severe case of central, centrifugal scarring alopecia
become pink and flattened, and an artifactual cleft between epithelium and
stroma is often found. In fact, the epithelium may appear to be floating within
the cleft (Figure 19.16). Although these later changes are often seen in patients
with long-standing LPP, they are not pathognomonic and can be observed in
other forms of inflammatory, scarring alopecia.
In time, the follicle is entirely destroyed. At first, retained hair shafts fragment
and a granulomatous response is observed, but eventually the follicles are
replaced by columns of connective tissue, giving the appearance of a burnt out
scarring alopecia (Figure 19.17; see also Chapter 22). As is the case in all forms
of inflammatory, scarring alopecia, the healing process may result in clinical and
histological polytrichia (Figure 19.18).
Grouped globular immunofluorescence (usually IgM), especially when found
adjacent to the follicular epithelium, is the characteristic pattern seen in LPP
(Figure 19.19). Linear deposits of immunoreactants are typical of lupus
erythematosus. This distinction can be important, because the scarring alopecia
of LPP and discoid lupus erythematosus may resemble each other both clinically
and histologically.
Figure 19.5 In this patient, lesions of lichen planopilaris have resulted in polytrichia
Because of poor site selection or sampling error, often the only changes found
are perifollicular fibroplasia and chronic inflammation. These changes are nonspecific and cannot be considered diagnostic. Additional biopsies are required to
establish the diagnosis whenever non-specific changes are found. However,
typical clinical lesions of lichen planus on other parts of the body strongly
support the diagnosis of LPP.
SUMMARY
Clinical correlation: various patterns of hair loss are seen, but most commonly
there are scattered foci of partial hair loss. Perifollicular erythema and scaling are
common to all cases. Lichen planus lesions elsewhere on the body support the
diagnosis of LPP on the scalp.
Histological findings:
Figure 19.6 The early changes of lichen planopilaris include a mononuclear cell
infiltrate obscuring the interface between epithelium and dermis. Vacuolar alteration of
the outermost layer of the outer root sheath is prominent in the early stages of the disease.
Original magnification 400
Figure 19.8 Transverse sections (see also Figures 19.9 and 19.10) taken at multiple levels
clearly demonstrate the depth of inflammation in lichen planopilaris. In this specimen,
inflammation is most intense at the level of the upper isthmus. Original magnification 40
Figure 19.10 Transverse section at the suprabulbar level, showing relatively sparse
inflammation. This is a typical pattern for lichen planopilaris. Original magnification 40
Elston DM, Bergfeld WF. Cicatricial alopecia (and other causes of permanent alopecia).
In Olsen EA, ed. Disorders of Hair Growth. New York:McGraw-Hill, 1994:
285314
Elston DM, McCollough ML, Warschaw KE, Bergfeld WF. Elastic tissue in scars and
alopecia. J Cutan Pathol 2000; 27: 14752
Headington JT. Cicatricial alopecia. Dermatol Clin 1996; 14:77382
Figure 19.11 This specimen from a patient with lichen planopilaris had typical follicular
changes but also interfollicular epidermal involvement. Vacuolar interface alteration,
numerous colloid bodies, and focal hypergranulosis can be seen. Original magnification
200
Figure 19.12 Another view of the specimen shown in Figure 19.11. Original
magnification 400
loannides D, Bystryn JC. Immunofluorescence abnormalities in lichen planopilaris.Arch
Dermatol 1992; 128:21416
Matta M, Kibbi AG, Khattar J, Salman SM, Zaynoun ST. Lichen planopilaris: a
clinicopathologic study. J Am Acad Dermatol 1990; 22:5948
Mehregan DA,Van Hale HM, Muller SA. Lichen planopilaris: clinical and pathologic
study of forty-five patients. J Am Acad Dermatol 1992; 27:93542
Silvers DN, Katz BE,Young AW. Pseudopelade of Brocq is lichen planopilaris: report of
four cases that support this nosology. Cutis 1993; 51:99105
Smith WB, Grabski WJ, McCollough ML, Davis TL Immunofluorescence findings in
lichen planopilaris: a contrasting experience. Arch Dermatol 1992; 128:14056
Sperling L, Solomon A, Whiting D. A new look at scarring alopecia. Arch Dermatol 2000;
136:23542
Thomsen HK. Lichen planopilaris or lupus? J Am Acad Dermatol 1999; 40:284
Waldorf DS. Lichen planopilaris. Histopathologic study of disease. Progression to
scarring alopecia. Arch Dermatol 1966; 93:68491
Whiting DA. Cicatricial alopecia: clinico-pathological findings and treatment. Clin
Dermatol 2001; 19:211 -25
Figure 19.13 Another view of the specimen shown in Figures 19.11 and 19.12. Original
magnification 400
Figure 19.15 Later stage lichen planopilaris, as in Figure 19.14. Original magnification
400
Figure 19.16 The more advanced changes seen in lichen planopilaris include prominent
artifactual clefting between epithelium and perifollicular connective tissue. Original
magnification 400
Figure 19.17 End-stage lichen planopilaris. The epithelium has been completely
destroyed. After the residual hair shaft has been resorbed, inflammation will subside and a
column of connective tissue will remain. Original magnification 400
Figure 19.18 In this end-stage specimen of lichen planopilaris, the follicular infundibula
of several adjacent follicles have merged (polytrichia) to form a single, enlarged ostium.
Original magnification 200
Figure 19.19 Immunofiuorescent staining for IgG in a specimen from a patient with
lichen planopilaris. Globular, clustered colloid bodies concentrated at the lower half of the
infundibulum are highlighted bright green. Linear staining of the epithelium is absent.
Slide courtesy of Kathleen David-Bajar, MD
CHAPTER 20
Frontal fibrosing alopecia
CHAPTER 21
Chronic cutaneous lupus erythematosus
(discoid lupus erythematosus)
Figure 21.1 Typical plaque of chronic cutaneous lupus erythematosus on the scalp of a
dark-skinned woman
Figure 21.2 Large plaque of chronic cutaneous lupus erythematosus centered on the
crown of the scalp. This pattern can easily be confused with central, centrifugal scarring
alopecia
Figure 21.3 Large patch of chronic cutaneous lupus erythematosus resembling alopecia
areata. In the center of the patch is a dyspigmented and slightly atrophic plaque that
serves as a clue to the correct diagnosis
Figure 21.4 Burnt out lesion of chronic cutaneous lupus erythematosus (CCLE),
indistinguishable clinically from Brocqs alopecia or from other forms of end-stage
scarring hair loss. This patient with mild, chronic systemic lupus erythematosus had an
earlier biopsy specimen taken from this site that was diagnostic of CCLE
(Figures 21.17 and 21.18). Globular deposits of IgM representing colloid bodies
may be present, but not as commonly as in LPP.
SUMMARY
Clinical correlation: The diagnosis of CCLE requires histological confirmation
and cannot be based solely on the clinical appearance of scalp lesions. These
may resemble classic DLE lesions, with alopecia, erythema, epidermal atrophy
and dilated, plugged follicular ostia. Central hypopigmentation and peripheral
hyperpigmentation are commonly seen in dark-skinned individuals. The
distribution and degree of clinical inflammation varies among patients, and
plaques of CCLE may result in patterns resembling alopecia areata, LPP, central,
centrifugal scarring alopecia and Brocqs pseudopelade.
Histological findings:
Figure 21.5 Vacuolar interface alteration of the follicular epithelium is a constant feature
of chronic cutaneous lupus erythematosus, but here and in Figure 21.6 the degree of
inflammation is scanty. Original magnification 200
Figure 21.6 Chronic cutaneous lupus erythematosus, with vacuolar interface alteration
and scanty inflammation, as in Figure 21.5. Original magnification 400
Figure 21.8 Chronic cutaneous lupus erythematosus, with dense inflammation. Original
magnification 200
Yell JA, Mbuagbaw J, Burge SM. Cutaneous manifestations of systemic lupus
erythematosus. Br J Dermatol 1996; 135:35562
Figure 21.11 Inflammation may extend down to the follicular bulbs. Original
magnification 40
Figure 21.12 Follicular stela infiltrated by copious plasma cells and lymphocytes.
Original magnification 400
Figure 21.13 Follicular stela infiltrated by copious plasma cells and lymphocytes.
Original magnification 400
Figure 21.14 An increase in interfollicular dermal mucin (here stained blue) is commonly
found in chronic cutaneous lupus erythematosus. Colloidal iron stain, original
magnification 400
Figure 21.15 Epidermal involvement in a scalp biopsy from a patient with chronic
cutaneous lupus erythematosus (CCLE). Hyperkeratosis with hypogranulosis and
epidermal thinning help to differentiate CCLE from lichen planopilaris. Original
magnification 200
Figure 21.16 Artifactual clefting between the follicular epithelium and the stroma can be
seen in this example of chronic cutaneous lupus erythematosus. Original magnification
200
Figure 21.17 Immunofluorescent staining for IgM in a patient with chronic cutaneous
lupus erythematosus. A bright, green, granular band of staining highlights the epidermal
and follicular basement membrane zones. Slide courtesy of Kathleen David-Bajar, MD
Figure 21.18 Gray-scale rendition of Figure 21.17 identifies the epidermal basement
membrane zone (EP B Z) and the follicular basement membrane zone (F B Z)
CHAPTER 22
Brocqs alopecia (pseudopelade of Brocq)
and burnt out scarring alopecia
BROCQS ALOPECIA
The term pseudopelade of Brocq is a source of much confusion and fruitless
debate, and should be abandoned. Pseudopelade has been used in recent decades
to describe some patients with central, centrifugal scarring alopecia (CCSA), a
very different condition from that described by Dr Brocq. To avoid confusion,
we can refer to the entity described by Brocq as Brocqs alopecia. Brocqs
alopecia is not a distinct disease but a clinical pattern of scarring alopecia. It is an
end-stage or clinical variant of several other forms of scarring alopecia and a
diagnosis of exclusion. The same pattern of hair loss can be seen in burnt out
lichen planopilaris (LPP), discoid lupus erythematosus (DLE) and other forms of
cicatricial hair loss. If a definitive diagnosis of DLE, LPP, CCSA or another form
of scarring alopecia can be made based on clinical, histological or
immunofluorescent features, then the term Brocqs alopecia cannot be used. If a
primary form of Brocqs alopecia exists, it has yet to be convincingly described.
The Brocqs alopecia pattern of hair loss is very uncommon. The typical
patient is a Caucasian adult who is surprised to discover discrete, asymptomatic
areas of scalp hair loss. In some patients, the disease is slowly progressive, and
new areas of alopecia develop over a period of months to years. However, the
condition often worsens in spurts, with periods of activity followed by dormant
periods. This is distinctly different from the slow but steady disease progression
seen in forms of CCSA described in Chapter 18. Disease progression in Brocqs
alopecia eventually terminates spontaneously.
Unlike CCSA, Brocqs alopecia results in irregularly shaped and often widely
distributed and grouped bald patches on the scalp. Cases with exclusive crown or
vertex involvement may actually represent examples of burnt out CCSA.
The individual lesion is hypopigmented (porcelain white is the classic
description) and slightly depressed (atrophic). Lesions are often irregularly
shaped, as opposed to the round or oval patches usually seen in alopecia areata
and most cases of CCSA (Figure 22.1). The classic description of footprints in
the snow refers to dermal atrophy causing a slight depression below the
surrounding normal scalp. In fact, many cases of Brocqs alopecia do not
Figure 22.1 Typical lesion of Brocqs alopecia in a woman whose hairdresser discovered
the bald spot. There was no clinical evidence of active inflammation, and the histological
findings were those of burnt out scarring alopecia
demonstrate atrophy. Usually only mild erythema and slight perifollicular scaling
are present, and often there is no clinical evidence of inflammation. In fact, some
authors have argued that any inflammation excludes Brocqs alopecia from the
clinical differential diagnosis. Just as in other forms of scarring alopecia, a few
isolated hairs may remain within an otherwise smooth, shiny, denuded patch.
The histological findings of Brocqs alopecia have yet to be clearly defined.
The criteria established by Pinkus in 1978 are not correlated in any way with
clinical features. Thus, pseudopelade as described by Pinkus is a histological
and not a clinical entity. In most cases of Brocqs alopecia, the active lesion is
elusive, and the typical histological findings are those of a burnt out scarring
alopecia (see below).
The histological findings of pseudopelade more recently described apply to a
subset of CCSA rather than Brocqs alopecia. It would not be surprising to find
an occasional case of Brocqs alopecia demonstrating the typical histological
findings of CCSA. Brocqs alopecia is, after all, the end stage of several
different forms of scarring alopecia. A prospective study of Brocqs alopecia
with sound clinical correlation has yet to be performed.
BURNT OUT SCARRING ALOPECIA
All too often, pathologists must render the diagnosis of a burnt out or end
stage scarring alopecia. The histological term burnt out scarring alopecia, like
the clinical term Brocqs alopecia, does not indicate a specific disease but a
pattern common to several entities. There are two possible reasons for finding
this pattern. First, the patients disease may, in fact, have truly burnt out like an
old forest fire, with no further follicular destruction. More commonly, however,
the end stage pattern is the result of clinicians unfortunate choice of biopsy
sites. Frequently, bald or nearly bald areas are sampled, instead of spots where
numerous hairs are still present. The advancing border of a zone of scarring
alopecia is always a more productive site than a bald zone. Areas with subtle
Figure 22.2 The paucity of terminal hairs, lack of inflammation and absence of sebaceous
glands are typical of a burnt out scarring alopecia. This particular example was taken
from a patient who proved to have central, centrifugal scarring alopecia (based on
additional specimens). Original magnification 40
Figure 22.3 Naked hair shaft surrounded by granulomatous inflammation and fibrosis.
Original magnification 400
Figure 22.4 Transverse section of columns of connective tissue that mark the sites of
former follicles (arrowhead). The absence of associated inflammation indicates that the
destructive process has truly burnt out, at least at the site chosen for biopsy. Original
magnification 100
biopsy site. However, this end stage histological finding indicates that the
patient has active, progressive disease.
Figure 22.5 Vertical section of a column of connective tissue, the site of a former follicle
(follicular scar). Original magnification 100
SUMMARY
Brocqs alopecia
Clinical correlation: usually in a Caucasian adult, who discovers discrete,
asymptomatic, hypopigmented, slightly depressed (atrophic) and irregularly
shaped bald patches affecting any portion of the scalp.
Histological findings: Brocqs alopecia represents the end stage of various
forms of scarring alopecia and usually shows features of a burnt out scarring
alopecia (see below). If typical histological findings of LPP, CCSA or other
types of scarring alopecia are found, the diagnosis of Brocqs alopecia can be
excluded.
Figure 22.6 Higher power view of the follicular scar shown in Figure 22.5. Original
magnification 200
Figure 22.7 Dense, acute and chronic inflammation surrounds a residual hair shaft (lost
during processing). This histological pattern often corresponds to the clinical finding of
pustules or crusted papules. These are late and non-specific findings. Original
magnification 200
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Brocq L, Lenglet E,Ayrignac J. Recherches sur lalopcie atrophiante, varit
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Pinkus H. Differential patterns of elastic fibers in scarring and non-scarring alopecias. J
Cutan Pathol 1978; 5:93104
Ronchese F. Pseudopelade. Arch Dermatol 1960; 82:33643 Silvers DN, Katz BE,Young
AW. Pseudopelade of Brocq is lichen planopilaris: report of four cases that support
this nosology. Cutis 1993; 51:99105
Sperling L, Solomon A, Whiting D. A new look at scarring alopecia. Arch Dermatol 2000;
136:23542
Templeton SF,Solomon AR. Scarring alopecia: a classification based on microscopic
criteria. J Cutan Pathol 1994; 21:97109 Whiting DA. Cicatricial alopecia: clinicopathological findings and treatment. Clin Dermatol 2001; 19:211 -25
CHAPTER 23
Acne keloidalis (folliculitis keloidalis)
Often called acne keloidalis nuchae, this disorder typically affects young Black
men and occasionally young Black women. Acne keloidalis (AK) is at least ten
times more common in Blacks than in Caucasians and comprises nearly 0.5% of
all dermatological cases in the Black population. However, the condition can
occur in Caucasian men and on rare occasions in Caucasian women. It begins as
small, smooth, firm papules with occasional pustules on the occipital scalp and
posterior neck. In a minority of cases, lesions are more numerous on the vertex
and crown, and so it is best to use the term acne keloidalis without the modifier
nuchae. Initially hairs can be seen exiting the papules, but the hair shafts are
soon shed. With time, the papules resolve and leave small zones of alopecia
within a field of papular lesions. In many patients, the papules coalesce and form
firm, hairless, keloid-like protuberant plaques (hence the term keloidalis) that can
be painful and cosmetically disfiguring. Abscesses and sinuses exuding pus can
be present in advanced cases. Although AK may be asymptomatic, mild
symptoms of burning and itching are often present.
The cause of AK remains unclear. The notion that lesions of AK are caused by
ingrowing hairs, analogous to the situation in pseudofolliculitis barbae, has
been disproved. AK is frequently found in association with central, centrifugal
scarring alopecia (CCSA), suggesting a common or related pathogenesis
(Figures 23.123.4). Occasionally, the papular lesions and hair loss of AK extend
up onto the vertex of the scalp, producing a clinical overlap of AK and CCSA.
HISTOLOGICAL FINDINGS
Most descriptions of the pathology of AK are based on late-stage, nodular
lesions. When early, papular lesions or samples of perilesional skin are studied,
the findings are those of a chronic, lymphocytic folliculitis. AK is a primary form
of scarring alopecia, and many of the histological findings closely resemble those
found in certain other forms of cicatricial alopecia. The most common findings,
in order of frequency found, are: perifollicular, chronic inflammation
(lymphocytic and plasmacytic), most intense at the level of the isthmus and
lower infundibulum (Figures 23.5 and 23.6); lamellar fibroplasia, most marked
at the level of the isthmus; complete disappearance of sebaceous glands
Figure 23.1 Typical acne keloidalis lesions on the nuchal region of a woman with typical
central, centrifugal scarring alopecia on the crown
Figure 23.2 Central, centrifugal scarring alopecia on the crown of the woman in
Figure 23.1
Figure 23.3 Acne keloidalis lesions on the nuchal region of a man with central,
centrifugal scarring alopecia on the vertex/ crown
Figure 23.4 Central, centrifugal scarring alopecia on the vertex/crown of the man in
Figure 23.3
focal bald spot. Others, however, can serve as a nidus for marked fibrosis,
persistent inflammation and bacterial superinfection. The resulting tissue
hypertrophy traps adjacent follicles, and eventually leads to the keloidal,
hypertrophic scarring seen in some patients with AK.
Even some specimens from normal-appearing perilesional skin may contain
true follicular scars, demon-strating subclinical disease. Special stains for
bacteria demonstrate relatively few organisms, suggesting that bacterial
overgrowth is not important in the pathogenesis of the disease.
Many of the histological features of AK are shared by CCSA. This overlap of
histological features suggests a common pathogenesis for AK and CCSA, a
notion that is supported by the frequent occurrence of both conditions in the
same patient. As mentioned above, clinical features sometimes overlap as well.
SUMMARY
Clinical correlation: small, smooth, firm follicular papules with occasional
pustules, eventually leading to partial hair loss; most common on the nuchal
Figure 23.5 Specimen taken from normal-appearing, perilesional skin in a patient with
typical acne keloidalis nuchae. Two follicles sharing a common infundibulum
demonstrate perifollicular lymphocytic inflammation, lamellar fibroplasia and loss of the
sebaceous glands. Two normal-appearing adjacent follicles (on the right side, also sharing
a fused infundibulum) are present for comparison. Original magnification 100
region (occipital scalp and posterior neck). With advanced disease, there are
coalescent papules forming firm, hairless, keloid-like protuberant plaques.
Histological findings:
Figure 23.6 Higher power view of the section shown in Figure 23.5. Original
magnification 200
Figure 23.7 In a specimen taken from a small papular lesion of acne keloidalis nuchae,
two follicles (sharing a fused infundibulum) are surrounded by dense, lymphocytic
inflammation. Original magnification 200
Figure 23.8 Specimen taken from a papular lesion of acne keloidalis nuchae shows dense
perifollicular chronic inflammation, most intense at the level of the lower isthmus. The
epithelium is disrupted at this level, and such follicles are doomed to destruction. Original
magnification 40
Figure 23.9 Higher power view of the specimen in Figure 23.8. Original magnification
100
Figure 23.10 When the epithelium of follicles has been disrupted or destroyed, the
inflammatory infiltrate is often rich in plasma cells as well as lymphocytes. Original
magnification 400
Figure 23.11 Two follicles in a papular lesion of acne keloidalis nuchae, sectioned at the
level of the upper isthmus. The epithelium of one follicle has been completely destroyed
below the level of the isthmus, which would result in permanent hair loss. Original
magnification 200
Figure 23.12 Three hairs in a papular lesion of acne keloidalis nuchae, sectioned at the
level of the lower isthmus. Original magnification 200
CHAPTER 24
Dissecting cellulitis of the scalp
(perifolliculitis capitis abscedens et
suffodiens)
the passage of time, inflammation extends throughout the dermis and invades the
follicular epithelium, and there is subsequent epithelial injury and follicular
destruction. Repetitive injury to the follicles results in acneiform dilatation of the
infundibula, with perifollicular neutrophilic inflammation (Figure 24.10).
If fluctuant nodules and sinuses are sampled, large perifollicular and mid- to
deep dermal abscesses composed of neutrophils, lymphocytes and often
numerous plasma cells are seen. Eventually, chronic abscesses become lined
with squamous epithelium derived from the overlying epidermis, and true sinus
tracts are formed. As follicles are completely destroyed, inflammation subsides
and is replaced by dense fibrosis of the dermis and the superficial fat.
SUMMARY
Clinical correlation: young adults, usually male and often African American,
with boggy nodules and a purulent discharge on the scalp. Lesions are scattered,
but most are concentrated on the crown, vertex and upper occiput.
Figure 24.4 Another section showing early lesions of dissecting cellulitis (see
Figure 24.3). Original magnification 40
Figure 24.5 A terminal catagen hair bulb is surrounded, but not invaded, by a dense
lymphocytic infiltrate. Original magnification 400
Histological findings:
Very early lesions are seldom evaluated, but may show moderately dense,
lymphocytic, perifollicular inflammation surrounding the lower half of the
follicle
Figure 24.6 In well-established dissecting cellulitis, the deep dermis and superficial fat
are replaced by a dense, mixed inflammatory infiltrate, edema and vascular proliferation.
Original magnification 200
Figure 24.8 Despite the loss of follicles and the severity of deep inflammation, sebaceous
glands persist well into the course of the disease. Original magnification 40
Figure 24.9 Dissecting cellulitis. Sebaceous glands are spared. Original magnification
100
CHAPTER 25
Tufted folliculitis
The tufting seen in this pattern of hair disease is common to several forms of
scarring alopecia (Figure 25.1). It is not a specific disease, but an end stage of
several different conditions. In spite of this, tufted folliculitis continues to be
applied as a diagnostic term in the medical literature. Such usage is incorrect and
misleading.
Tufting occurs because the infundibular epithelium of damaged follicles often
heals with the formation of a large, common infundibulum (Figure 25.2). A zone
of fibrosis separates individual tufts. Another name for this phenomenon is
polytrichia. Although tufting is fairly common in cases of central, centrifugal
scarring alopecia, it can occasionally be seen in a wide variety of
233
Figure 25.2 A histological view of polytrichia (tufting). Whenever the upper halves of
adjacent follicles become inflamed and damaged, a large, common infundibulum may
form. Original magnification 100
Powell JJ, Dawber RP, Gatter K. Folliculitis decalvans including tufted folliculitis:
clinical, histological and therapeutic findings. Br J Dermatol 1999140:32833
Sperling L, Solomon A, Whiting D. A new look at scarring alopecia. Arch Dermatol 2000;
136:23542
CHAPTER 26
Tinea capitis
Most cases of tinea capitis (fungal infection of scalp hair) seen in North America
are caused by Trichophyton tonsurans, a large-spore endothrix infection.
Microsporum canis (ectothrix) is also found occasionally. Different species may
be more prevalent in other parts of the world. The fungus invades and multiplies
within the hair shaft below the surface of the skin, causing hair fragility and
breakage. This results in bald spots with follicular ostia filled with keratinous
debris or black dots, the residua of infected, pigmented hair shafts
(Figure 26.1). Variable degrees of inflammation may be seen, ranging from none
(no erythema or scaling) to highly inflamed, purulent, edematous and crusted
plaques (kerion; Figure 26.2). Pustular lesions on the scalp should always raise
suspicion for tinea capitis. Tinea capitis almost always affects children, and
African American children seem to be especially prone to infection. Any
inflamed or scaly bald spot on the scalp of an African American child should be
considered to be tinea capitis unless proven otherwise. However, adults of all
races (but especially African American women) can also be affected. If treated
early, tinea capitis heals without scarring or hair loss. However, highly
inflammatory lesions or untreated lesions may eventuate in permanent hair loss.
Inflammatory tinea capitis is one of several causes of tufted folliculitis
(polytrichia).
HISTOLOGICAL FINDINGS
The common denominator and pathognomonic finding in all cases of tinea
capitis is the presence of spores (and sometimes hyphae) within the hair shaft
(Figures 26.3 and 26.4). Only a few follicles within a specimen may demonstrate
this finding, so that transverse sectioning is the most reliable way to establish the
diagnosis. The histological degree of inflammation is highly variable. In many
cases, hairs packed with spores are almost free of perifollicular inflammation
(Figure 26.5). A superficial and/or deep perifollicular infiltrate rich in
neutrophils, eosinophils, lymphocytes and plasma cells may be present (Figures
26.626.9).
Exceptional cases of inflammatory tinea capitis resembling dissecting
cellulitis (Figure 26.10) have a deep, dense lymphocytic infiltrate that surrounds
Figure 26.1 Nine-month-old African American baby with black dot tinea capitis caused
by Trichophyton tonsurans. Although there is extensive hair loss, clinical inflammation
(erythema, scaling) is not present
hair bulbs and extends into the fat (Figures 26.1126.14). Suprisingly, although
clinical findings and degree of histological inflammation are quite dramatic,
infected follicles may be difficult to find. In these cases, fungal cultures may be
required to establish the diagnosis.
SUMMARY
Clinical correlation: patients are most often children with bald, scaly,
erythematous patches on the scalp. Dark and widened follicular ostia (black
dots) may be present. The clinical manifestations of inflammation are highly
variable and range from mild scaling without erythema to intense erythema,
induration, pustules and a purulent discharge.
Histological findings:
Spores (and sometimes hyphae) within hair shafts are a diagnostic feature, but
not every follicle in a specimen may be involved
The amount of perifollicular inflammation is highly variable
Figure 26.3 Transverse section of follicle infected with fungal spores.An affected shaft
(right side) is adjacent to one that is totally spared. Original magnification 400
Figure 26.4 Vertical section of follicle infected with fungal spores. Original
magnification 400
Figure 26.5 Same follicles as in Figure 26.3. Although one hair shaft is filled with spores,
perifollicular inflammation is scanty. Original magnification 200
Figure 26.6 An example of highly inflammatory tinea capitis. Such cases are often
associated with pustules or a purulent discharge and crusting. Original magnification 40
Figure 26.8 Inflammation often extends into the follicular wall, and in some cases
eventuates in follicular destruction. Original magnification 400
Figure 26.9 The deep portion of the infiltrate seen in tinea capitis is predominantly
composed of lymphocytes and plasma cells. Original magnification 400
Figure 26.10 Tinea capitis resembling dissecting cellulitis in an adolescent boy. A culture
identified the causative organism as Trichophyton tonsurans
Figure 26.11 Tinea capitis resembling dissecting cellulitis (same adolescent boy as in
Figure 26.10). Inflammation is intense but quite deep, predominantly involving the
superficial fat. Original magnification 40
Figure 26.13 Hairs adjacent to the zone of inflammation may be converted to the catagen/
telogen phase. Original magnification100
Figure 26.14 Even anagen hairs may remain unaffected although surrounded by a sea of
inflammation. Original magnification 400
CHAPTER 27
Aplasia cutis congenita of the scalp
Aplasia cutis congenita (ACC) is the congenital absence of skin. The scalp is by
far the most common site for solitary lesions of ACC. Occasionally two or even
three lesions of ACC can occur together on the scalp. Lesions are often located
near the hair whorl on the vertex of the scalp, and can be various shapes and
sizes (0.510 cm). At the time of birth the defects may be deeply ulcerated,
superficially eroded or completely healed but scarred (Figure 27.1).
HISTOLOGICAL FINDINGS
Biopsy specimens from patients with ACC are often obtained well after birth,
when the lesions are excised for cosmetic reasons. The epidermis usually
demonstrates some flattening of the rete ridges. A few
Figure 27.1 Solitary lesion of aplasia cutis congenita adjacent to the scalp whorl of a
young boy. The child had been born with a friable wound on the scalp that healed with
this scarred appearance
residual hair follicles may remain stranded in a dermis that is otherwise devoid
of adnexal structures, including follicles and sweat glands. When the central
portion of the lesion is compared with the surrounding normal scalp skin, the
dermis usually appears thickened, because the superficial subcutaneous fat has
been replaced by collagenous tissue (Figures 27.227.4). However, in some
instances, the dermis is relatively thinned.
Figure 27.2 Dermal thickening and loss of follicles is evident in the central portion (right
side) of this lesion of aplasia cutis congenita as compared with the border (left side).
Original magnification 40
Figure 27.3 Flattening of the rete ridges is evident in the central portion of this lesion of
aplasia cutis congenita (right side) as compared with the border (left side). Loss of follicles
and thickening of the dermis are also seen. Original magnification 40
Collagen bundles are usually thickened and sclerotic in appearance, and are
often oriented in bundles parallel to the skin surface, as would be expected in a
well-healed scar (Figures 27.5 and 27.6). Occasionally, however, the thickened
dermal collagen appears deceptively normal, because of the random orientation
of collagen bundles. However, even in these cases, adnexal structures are absent
Figure 27.5 Sclerotic, parallel collagen bundles in a lesion of aplasia cutis congenita.
Original magnification 40
or rare (Figures 27.7 and 27.8). Inflammation is not seen in completely healed
lesions of ACC.
Figure 27.7 In this lesion of aplasia cutis congenita, the dermis is relatively thinned.The
loose, random orientation of collagen bundles looks normal, but adnexal structures are
sparse. Original magnification 40
SUMMARY
Clinical correlation: a single (rarely multiple) hairless plaque, most often located
near the hair whorl on the vertex of the scalp. Lesions are present at birth, and
can be of various shapes and sizes. At birth, the defects may be deeply ulcerated,
superficially eroded or completely healed but scarred. Most lesions are excised
well after birth, when they are completely healed.
Histological findings:
Deeken JH, Caplan RM. Aplasia cutis congenita. Arch Dermatol 1970; 102:3869
Frieden IJ. Aplasia cutis congenita: a clinical review and proposal for classification. J Am
Acad Dermatol 1986; 14:64660
Itin P, Pletscher M. Familial aplasia cutis congenita of the scalp without other defects in 6
members of three successive generations. Dermatologica 1988; 177:1235
Kenner JR, Sperling LC. Pathological case of the month. Temporal triangular alopecia
and aplasia cutis congenita. Arch Pediatr Adolesc Med 1998; 152:12412
Kosnik EJ, Sayers MP. Congenital scalp defects: aplasia cutis congenita. J Neurosurg
1975; 42:326
Munkvad JM, Nielsen AO, Asmussen T. Aplasia cutis congenita. A follow-up evaluation
after 25 years. Arch Dermatol 1981; 17:2323
Sargent LA Aplasia cutis congenita of the scalp. J Pediatr Surg 1990; 25:121113
Stephan MJ, Smith DW, Ponzi JW, Alden ER. Origin of scalp vertex aplasia cutis. J
Pediatr 1982; 101:8503
CHAPTER 28
Overview of hair shaft disorders
A detailed review of hair shaft abnormalities is beyond the scope of this text.
Several excellent reviews of hair shaft disorders are available and are listed at the
end of the appropriate following sections. However, pathologists,
dermatopathologists and dermatologists are sometimes called upon to identify a
hair shaft disorder from a submitted sample of hair. This section will focus on
describing the morphological features of shaft disorders as seen by light
microscopy.
TRICHORRHEXIS NODOSA
The basic cause of trichorrhexis nodosa is trauma to the hair shafts, but any
inherent weakness of the shaft may result in this defect. All the various forms of
weathering (grooming, washing, styling, etc.) can cause or worsen the disorder.
Examples of physical trauma include excessive brushing, the application of heat,
hair pulling in trichotillomania and scratching due to pruritic scalp disorders.
Examples of chemical trauma include permanent straightening (relaxing;
Figure 28.1), permanent waving, dyeing and shampooing. Clinically,
trichorrhexis nodosa may appear as small, white or gray specks on the hair shafts.
Affected hairs are susceptible to fracture, which can result in patchy or diffuse
alopecia, depending on the extent of involvement. Although scalp hair is usually
affected, trichorrhexis nodosa can be found on pubic hair and other hairy areas of
the body.
The nodes of trichorrhexis nodosa represent foci of frayed cortical fibers that
bulge out through a ruptured cuticle. Scanning electron microscopy has shown
that the earliest change is a focal loss of cuticular cells, which eventually results
in hair fiber separation and fracture. Each node then takes on the appearance of
two paintbrushes whose bristles have been pushed together (Figures 28.2 and
28.3). Each bristle represents a cornified cortical cell, which can separate from
adjacent cells once the binding function of the cuticle is impaired.
Trichorrhexis nodosa is often found in association with other types of hair
shaft abnormalities caused by weathering or inherited hair fragility. Therefore,
trichoclasis (greenstick fractures), trichoptilosis (split ends) and trichoschisis
Figure 28.1 Hair loss in an African American woman caused by chemical relaxers.
Longer hairs along the margins of the bald spot showed dramatic trichorrhexis nodosa
of the proximal shafts. Hair loss was due to fractures of the damaged shafts
(see below) are often found in hairs from the same patient or even the same shaft
(Figure 28.4).
BIBLIOGRAPHY
Caserio RJ. Diagnostic techniques for hair disorders. Part I: Microscopic examination of
the hair shaft. Cutis 1987; 40: 26570
Dawber RP. An update of hair shaft disorders. Dermatol Clin 1996; 14:75372
Rogers M. Hair shaft abnormalities: Part 1. Australas J Dermatol 1995; 36:17984
Whiting DA. Structural abnormalities of the hair shaft. J Am Acod Dermotol 1987; 16:
125
TRICHOSCHISIS
The term trichoschisis is used for a clean, transverse fracture across the hair
shaft through cuticle and cortex. A localized absence of cuticle cells can be
found at the site of the fracture. Although trichoschisls is occasionally found in
normal hair, it usually occurs in the setting of trichothiodystrophy (see below).
Figure 28.2 Focal loss of the hair cuticle results in cortical fiber separation and the fragile
nodes of trichorrhexis nodosa
Figure 28.4 Trichoclasis (greenstick fracture of hair) and trichorrhexis nodosa are often
found in the same weathered shaft
TRICHOTHIODYSTROPHY
Patients with trichothiodystrophy have congenitally brittle hair with low sulfur
content. This hair defect serves as the common denominator for patients with
neuroectodermal abnormalities and mutations in the xeroderma pigmentosum
XP-B and XP-D helicase subunits of the dual functional DNA repair/basal
transcription factor TFIIH. Depending on the precise mutation, patients may
Figure 28.8 Alternating light and dark banding (tiger tail phenomenon) in a patient with
trichothiodystrophy. A polarized hair with the filters in the crossed position is shown
here
Figure 28.9 The same shaft as in Figure 28.8, without filters in place
BIBLIOGRAPHY
Cleaver JE, Thompson LH, Richardson AS, States JC. A summary of mutations in the UVsensitive disorders: xeroderma pigmentosum, Cockayne syndrome, and
trichothiodystrophy. Hum Mutat 1999; 14:922
Figure 28.10 Nethertons syndrome. The distal portion of this hair shaft (left side) has
sunken into the proximal portion to create a node of trichorrhexis invaginata
Coin F, Marinoni JC, Rodolfo C, Fribourg S, Pedrini AM, Egly JM. Mutations in the XPD
helicase gene result in XP and TTD phenotypes, preventing interaction between XPD
and the p44 subunit of TFIIH. Nature Genet 1998; 20:1848
Dawber RP. An update of hair shaft disorders. Dermatol Clin 1996; 14:75372
Itin PH, Sarasin A, Pittelkow MR.Trichothiodystrophy: update on the sulfur-deficient
brittle hair syndromes. J Am Acod Dermatol 2001; 44:891920
Price VH. Trichothiodystrophy: update. Pediatr Dermatol 1992; 9:36970
TRICHORRHEXIS INVAGINATA
This rare, congenital hair shaft abnormality is seen in Nethertons syndrome, the
combination of short, brittle hair and an ichthyosiform erythroderma (especially
ichthyosis linearis circumflexa). Atopic dermatitis is also found in the majority
of patients. The defective gene in Nethertons syndrome is SPINK5, which
encodes a serine protease inhibitor.
Also called bamboo hair, trichorrhexis invaginata appears as multiple small
swellings spaced along the shaft at irregular intervals. Each swelling consists of a
cup-like expansion of the proximal hair cortex that surrounds a rounded distal
fragment, giving the defect the appearance of a ball-and-socket joint
(Figure 28.10). It appears as if the distal segment has intussuscepted down into
the proximal segment. These nodes are extremely fragile and susceptible to
fracture (Figures 28.11 and 28.12), and consequently the hair of affected persons
is short, thin and friable.
BIBLIOGRAPHY
Bitoun E, Chavanas S, Irvine AD, et al. Netherton syndrome: disease expression and
spectrum of SPINK5 mutations in 21 families. J Invest Dermatol 2002; 118:35261
Dawber RP. An update of hair shaft disorders. Dermotol Clin 1996; 14:75372
Whiting DA. Structural abnormalities of the hair shaft. J Am Acod Dermotol 1987; 16:
125
PILI ANNULATI
Also called ringed hair, pili annulati is characterized clinically by alternating
light and dark bands of color along the hair shafts. The colors are reversed when
the hairs are viewed by light microscopy. The banded appearance is obvious only
in blond or lightly pigmented hair, which often has a sandy appearance. The
condition may be present at birth or appear during infancy. Hair growth is
normal and in the majority of cases the hair is strong and sound, but there may be
some degree of shaft fragility. Trichorrhexis nodosa-like fractures may be
produced in the dark bands. There are no other associated abnormalities and
treatment is not required. The condition appears to be caused by a defect in hair
cornification.
The cortex of the abnormal bands contains zones of air-filled spaces
(Figure 28.13), between cortical fibers and within cortical cells. Medullary cells
are not involved in the process. The air-filled spaces can be confined to the
central portion of the shaft, or may involve the full thickness with associated
cuticle disruption.
Pili annulati has been associated with alopecia areata on more than one
occasion. It is not known whether or not this is pure coincidence.
BIBLIOGRAPHY
Dini G, Casigliani R, Rindi L, Grappone C, Melli MC, Lotti T.Pili annulati. Optical and
scanning electron microscopic studies. Int J Dermatol 1988;27:2567
Gummer CL, Dawber RP. Pili annulati: electron histochemical studies on affected hairs.
Br J Dermatol 1981; 105:3039
Ito M, Hashimoto K, Sakamoto F, Sato Y, Voorhees JJ. Pathogenesis of pili annulati.
Arch Dermatol Res 1988; 280:30818
Moffitt DL, Lear JT, de Berker DA, Peachey RD. Pili annulati coincident with alopecia
areata. Pediatr Dermatol 1998; 15:2713
Price VH,Thomas RS,Jones FT. Pili annulati. Optical and electron microscopic studies.
Arch Dermatol 1968; 98:6407
Smith SR, Kirkpatrick RC, Kerr JH, Mezebish D.Alopecia areata in a patient with pili
annulati. J Am Acad Dermatol 1995; 32:81618
BUBBLE HAIR
This distinctive hair shaft abnormality is usually seen in young women with a
localized area of uneven, fragile hairs. The involved hair is straighter and stiffer
than normal. Light microscopy has demonstrated that the hair shafts contain
large, irregularly spaced bubbles that expand and thin the hair cortex
(Figure 28.14). Hair fractures occur at the site of larger bubbles.
The problem is caused by traumatic hair care techniques involving heat, as
from a malfunctioning hair dryer. Once the damaged hair is trimmed, the
condition resolves completely with gentle hair styling.
BIBLIOGRAPHY
Detwiler SP, Carson JL,Woosley JT, Gambling TM, Briggaman RA. Bubble hair. Case
caused by an overheating hair dryer and reproducibility in normal hair with heat. J
Am Acad Dermatol 1994; 30:5460
Elston DM, Bergfeld WF, Whiting DA, et al. Bubble hair. J Cutan Pathol 1992; 19:
4394
Gummer CL Bubble hair: a cosmetic abnormality caused by brief, focal heating of damp
hair fibres. Br J Dermatol 1994; 131:9013
MONILETHRIX
Patients with monilethrix (beaded hair) have extremely brittle, beaded hairs
that emerge from keratotic, follicular papules. Hair shafts rarely grow more than
23 cm. The hairs fracture easily, usually resulting in severe alopecia. The
course is variable and seasonal, and some patients may show improvement in
adult life, while others seem to worsen. The condition usually appears in early
childhood, predominantly on the occiput and nape, but it can affect the entire
Figure 28.15 In monilethrix, the nodes correspond to the normal caliber of the hair and
the defective portion resides in the constrictions
scalp. Occasionally the child is born bald, with beaded hairs appearing several
months or years later. Facial and body hair are also involved in severe cases.
Monilethrix is usually inherited as an autosomal dominant trait with high
penetrance but variable expressivity. Recessive phenotypes have also been
described, as have sporadic cases. Mutations in two of the 11 known hair
keratins have been associated with monilethrix. Mutations in the hHb6 gene are
most common, but mutations in other genes (e.g., hHb1) can lead to the disease.
Hair shafts in patients with monilethrix show characteristic, evenly spaced,
elliptical nodes that are 0.71 mm apart (Figure 28.15). The intervals between
nodes will vary in different hairs from the same patient. Not every hair follicle is
affected in a synchronized fashion. The segments of hair shaft between nodes are
non-medullated, tapered constrictions. Imbricated cuticular scales are present on
the nodes, whereas the internodes show abnormal longitudinal ridging and often
absent scales. Scanning electron microscopy has demonstrated that the nodes
correspond to the normal caliber of the hair and that the defective portion resides
in the constrictions.
The hair roots have an architecture that conforms to the shaft abnormality,
with alternating constrictions, so that the hair is deformed as it is being
produced. Matrix cells destined to become cortical cells are particularly affected.
This seems to result in a decrease in number of cortical cells and thinning of the
hair shaft.
BIBLIOGRAPHY
Dawber RR An update of hair shaft disorders. Dermotol Clin 1996; 14:75372
Korge BP, Healy E, Munro CS, et al. A mutational hotspot in the 2B domain of human hair
basic keratin 6 (hHb6) in monilethrix patients. J Invest Dermotol 1998; 111:8969
Whiting DA. Structural abnormalities of the hair shaft. J Am Acad Dermotol 1987; 16:
125
PILI TORTI
Pili torti is the most misdiagnosed structural hair shaft defect, and it is often
confused with monilethrix when studied with light microscopy. Hairs in pili torti
are flattened and twisted on their longitudinal axes (Figures 28.16 and 28.17).
Each twist may be 90, 180, or up to 360. Typically, runs of four or five
twists are found at irregular intervals along the hair shaft. Involved hairs are
brittle, break off easily, do not achieve normal length and often have a spangled
or beaded appearance. The hairs are fragile and usually fracture within the
twists. The fractured distal tip often shows longitudinal fraying (trichoptilosis).
The hair follicles show no histological abnormalities other than some curvature
and twisting. Although it can be present at birth or emerge after puberty, pili torti
classically appears in early childhood when normal scalp hairs are replaced by
brittle, spangled hairs, especially in the occipital and temporal regions. The
spangled appearance is due to unequal reflection of light from the twisted
surface. The eye-brows and eyelashes may also be involved. There is a variable
degree of hair fragility ranging from patchy alopecia with coarse stubble to hairs
of 5 cm or more. Pili torti may improve after puberty or may persist throughout
life.
Pili torti is also found in various syndromes, such as Menkes kinky hair
disease, Bjornstad syndrome and Rapp-Hodgkin ectodermal dysplasia. The
numerous other distinctive features of these syndromes allow for proper
diagnosis, with pili torti offering an additional clue.
BIBLIOGRAPHY
Dawber RR An update of hair shaft disorders. Dermatol Clin 1996; 14:75372
Richards KA, Mancini AJ. Three members of a family with pili torti and sensorineural
hearing loss: the Bjornstad syndrome. J Am Acad Dermatol 2002; 46:3013
Whiting DA. Structural abnormalities of the hair shaft. J Am Acad Dermatol 1987; 16:
125
PILI MULTIGEMINI
This condition is typically found in the beard area, especially along the jaw.
Anywhere from two to eight hair matrices, each with its own papilla and separate
inner root sheath (Figure 28.18), form clusters of shafts that emerge from a
single follicular canal. Surrounding the cluster of hair bulbs is a common outer
root sheath that separates to invest each ascending hair shaft (Figures 28.19 and
28.20).
The shafts have various shapes in cross-section (flat, ovoid, triangular or
grooved), which probably results from the pressure between hairs in the same
follicle (Figure 28.21). Usually the condition is totally benign, but it may be
associated with perifollicular erythema resembling folliculitis. There is no
specific treatment for pili multigemini, which tends to be persistent. The
abnormal hairs will simply regrow after plucking.
BIBLIOGRAPHY
Cambiaghi S, Barbareschi M, Cambiaghi G, Caputo R. Scanning electron microscopy in
the diagnosis of pili multigemini. Acta Derm Venereol 1995; 75:1701
Mehregan AH.Thompson WS. Pili multigemini. Report of a case in association with
cleidocranial dysostosis. Br J Dermatol 1979; 100:31522
Pinkus H. Multiple hairs (Flemming-Giovannini): report of two cases of pili multigemini
and discussion of some other anomalies of the pilary complex. J Invest Dermatol
1951; 17:291 -301
261
Figure 28.18 Forcibly plucked hair from a follicle demonstrating pili multigemini. A
syncytium of outer root sheath envelops several separate matrices, each producing its own
shaft
The hair shafts in uncombable hair are usually triangular in cross-section, but
can be kidney-shaped, flat, or irregular. Most of the hair shafts will demonstrate
the irregular, triangular shape. Longitudinal grooves running along the entire
shaft are common, hence the name pili trianguli et canaliculi. Although grooving
of a few hair shafts can be found on the normal scalp, in the uncombable hair
syndrome at least 50% of hair shafts demonstrate this abnormality. Grooves can
often be seen with the light microscope (Figure 28.22), although they are best
visualized with the scanning electron microscope (Figure 28.23). Scalp biopsy
reveals that the inner root sheath has the same configuration as the shaft.
BIBLIOGRAPHY
Dupre A, Bonafe JL, Litoux F, Victor M. Uncombable hair syndrome. Pili trianguli et
canaliculi. Ann Dermatol Venereol 1978; 105:62730
Hicks J, Metry DW, Barrish J, Levy M. Uncombable hair (cheveux incoiffables, pili
trianguli et canaliculi) syndrome: brief review and role of scanning electron
microscopy in diagnosis. Ultrastruct Pathol 2001; 25:99103
262
Figure 28.19 Vertical section through the bulb of a follicle demonstrating pili multigemini.
A common outer root sheath invests the clustered sibling hairs
McCullum N, Sperling LC, Vidmar D. The uncombable hair syndrome. Cutis 1990; 46:
47983
263
Figure 28.20 Transverse section through the bulb of a follicle demonstrating pili
multigemini. There are several sub-papillae, each with its own matrix and inner root
sheath
Figure 28.21 Transverse section through the infundibulum. The clustered shafts have
angulated, bizarre shapes
264
Figure 28.22 The prominent grooving seen in the uncombable hair syndrome is visible
even with light microscopy
Figure 28.23 The grooving seen in the uncombable hair syndrome is best visualized
with scanning electron microscopy
Figure 28.24 Gently pulling on the hair was sufficient to remove numerous pencil point
hairs from a patient receiving systemic chemotherapy for a malignancy
BIBLIOGRAPHY
Brunner MJ, Facq LM. A pseudoparasite of the scalp hair. Arch Dermatol 1957; 75:583
Kligman AM. Hair casts. Arch Dermatol 1957; 75:50911
Scott MJ, Roenigk HH Jr. Hair casts: classification, staining characteristics, and
differential diagnosis. J Am Acad Dermatol 1983; 8:2732
265
Figure 28.25 A tapered hair gently pulled from the scalp of a patient with rapidly worsening
alopecia areata
Figure 28.26 A peripilar cast composed of infundibular keratin. This is the most common
kind of hair cast
266
Figure 28.27 Peripilar casts composed of distal fragments of inner root sheath. These casts
stain a dark blue with toluidine blue
Index
apoptosis in 66
catagen/telogen proportion in 30
cf. syphilitic alopecia 80
chronic 70, 71
exclamation mark hairs in 64
follicle density in 28
follicular dropout with intact sebaceous
gland 32
hair density and 29
hair loss patterns
circumscribed 63
diffuse 63
partial 63
reticular 63
inflammation in 30, 68
miniaturization in 71
mononuclear cell inflammatory
infiltrate 65
nanogen hairs in 71, 72, 73, 74
of beard area 63
pencil point hairs 6
subacute 68
suprabulbar zone in 67
trichomalacia in 32
alopecia totalis, stela in 77
alopecia totalis in evolution 63, 64, 65
anagen arrest 69, 149
anagen effluvium 69
anagen hair 1
African American 6
anatomy of 2
forcibly plucked 11
infundibulum of 4, 5
isthmus of 3
loose 87
newly growing 10
267
pull/pluck findings 10
suprabulbar zone 3
terminal type 2
anatomy
anagen hair 2
catagen hair 4
follicular in trichotillomania 47
normal 113
telogen hair 5
androgenetic alopecia 3742
cf. central centrifugal scarring alopecia
92
early stage appears as telogen effluvium
43
follicular dropout with intact sebaceous
gland 32
hair density and 28, 29
histological findings in 37
pattern of hair loss in 34
typical features of 37, 41
antibiotics, use in central centrifugal
scarring alopecia 93
anticoagulants 43
anticonvulsants 43
antithyroid drugs 43
aplasia cutis congenita of the scalp 137
139
apoptosis
in alopecia areata 66
in catagen hair 4
architecture, normal 113
arrector pili, insertion of 3, 5
axillary hair loss 101
-blockers 43
bacteria, staphylococci in central
centrifugal scarring alopecia 98
bamboo hair 144
beaded hair 146
beard
alopecia areata in 63
pili multigemini in 147
biopsy
Caucasian cf. African 21
checklist for description of 19
technique for taking 15
template for hair biopsy report 23
INDEX 269
INDEX 271
matrix 2
in alopecia areata 67
Menkes kinky hair disease 147
metabolic disturbance and telogen
effluvium 43
Microsporum canis 133
miniaturization
in androgenetic alopecia 38, 41
in chronic alopecia areata 71
in non-scarring alopecia in systemic
lupus erythematosus 83
in telogen effluvium 45
in temporal triangular alopecia 59
miniaturization of hair 29
minoxidil 39
monilethrix 146
mononuclear cell infiltrate,
in lichen planopilaris 102
in non-scarring alopecia in systemic
lupus erythematosus 83
morphea 26
mucin
dermal levels in chronic cutaneous
lupus erythematosus 111, 113
dermal levels in non-scarring alopecia
in systemic lupus erythematosus 84, 86
nanogen hair 71, 72, 73, 74
anagen-like 73
catagen-like 73
necrobiosis lipoidica 26
necrosis
in alopecia areata 68
of fat in postoperative alopecia 56
Nethertons syndrome 144
nodules in dissecting cellulitis 126
non-scarring alopecia in systemic lupus
erythematosus 8386
nuchal acne keloidalis 120
nutritional deficiency and telogen
effluvium 43
onion skin-like fibroplasia 95
papilla 2
papules
in acne keloidalis 119
INDEX 273
premature desquamation 32
of inner root sheath 93
pressure-induced alopecia in trichomalacia
32
processing of hair specimens 15
pseudofolliculitis barbae 119
pseudonits 151
pseudopelade 31, 91
pubic hair, loss of 101
pull/pluck findings,
anagen hair 10
catagen hair 10
dysmorphic 1013
snapped-off shafts 13
telogen hair 11
punch biopsy 15
pustules
in acne keloidalis 119
in central centrifugal scarring alopecia
92
in tinea capitis 134
radiation therapy, pencil point hairs 6
Rapp-Hodgkin ectodermal dysplasia 147
retinoids 43
telogen effluvium and 45
ringed hair 144
root
fibrous sheath 2
inner sheath 3
outer sheath 3
premature desquamation of inner
sheath 32
sampling, see hair specimens
sarcoidosis 25, 26
scalp
aplasia cutis congenita of 137139
appearance in alopecia areata 63
dissecting cellulitis of 125129, 125
129
normal adult 1
normal African American 22
normal Caucasian 22
normal in transverse section 20
sclerosis, in aplasia cutis congenita 138
sebaceous gland
well-established 82
systemic lupus erythematosus,
catagen/telogen proportion in 30
inflammation in 30
non-scarring alopecia in 8386
systemic lupus erythematosus, telogen
effluvium in 44
tapered hairs 149
telogen count
determination of 21, 22
in dissecting cellulitis 127
in loose anagen hair syndrome 87, 88
in non-scarring alopecia in systemic
lupus erythematosus 83
in postoperative alopecia 55
in subacute alopecia areata 70
in telogen effluvium 44
in traction alopecia 52
increase in androgenetic alopecia 39
telogen effluvium 33, 34, 4346
catagen/telogen proportion in 30
causes of 43
hair density and 28
in neonates 43
in systemic lupus erythematosus 44
syphilitic 79
telogen germ unit 5
telogen hair 1
anatomy of 5
early 9
forcibly plucked 10, 12
increased proportion 29
numbers in trichotillomania 48
percentage of as diagnostic feature 21
pull/pluck findings 11
pulling of 6
terminal cf. vellus 9
temporal triangular alopecia 5961
hair density and 29
miniaturization in 59
terminal hair
anagen phase 2
cf. vellus hair 1
cf. vellus hair in traction alopecia 53
cf. vellus in telogen phase 9
ratio to vellus hair 23
INDEX 275